Mitochondrial dysfunction in ALS (Q37914353)

25 August 2017

1 reference

title

Mitochondrial dysfunction in ALS (English)

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

amyotrophic lateral sclerosis

9 December 2024

main subject

1 reference

1 reference

1

Mauro Cozzolino

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A21827820

9 December 2024

2

Maria Teresa Carrì

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A21827820

9 December 2024

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=21827820

9 December 2024

publication date

2 August 2011

1 reference

full work available at URL

25 August 2017

https://api.elsevier.com/content/article/PII:S0301008211000918?httpAccept=text/xml

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

https://api.elsevier.com/content/article/PII:S0301008211000918?httpAccept=text/plain

9 December 2024

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

9 December 2024

published in

Progress in Neurobiology

1 reference

25 August 2017

volume

97

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25 August 2017

issue

2

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25 August 2017

page(s)

54-66

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Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis

25 August 2017

cites work

1

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

N-acetyl-L-cysteine improves survival and preserves motor performance in an animal model of familial amyotrophic lateral sclerosis

9 December 2024

2

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation.

9 December 2024

3

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis

9 December 2024

4

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial damage due to SOD1 deficiency in SH-SY5Y neuroblastoma cells: a rationale for the redundancy of SOD1

9 December 2024

5

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The ultrastructure of intramuscular nerves in amyotrophic lateral sclerosis

9 December 2024

6

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Increased motoneuron survival and improved neuromuscular function in transgenic ALS mice after intraspinal injection of an adeno-associated virus encoding Bcl-2.

9 December 2024

7

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization

9 December 2024

8

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Coenzyme Q10 as a possible treatment for neurodegenerative diseases

9 December 2024

9

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

A review of the neural mechanisms of action and clinical efficiency of riluzole in treating amyotrophic lateral sclerosis: what have we learned in the last decade?

9 December 2024

10

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Lost in translation: treatment trials in the SOD1 mouse and in human ALS.

9 December 2024

11

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Lessons from models of SOD1-linked familial ALS.

9 December 2024

12

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Transgenic SOD1 G93A mice develop reduced GLT-1 in spinal cord without alterations in cerebrospinal fluid glutamate levels

9 December 2024

13

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial dysfunction due to mutant copper/zinc superoxide dismutase associated with amyotrophic lateral sclerosis is reversed by N-acetylcysteine.

9 December 2024

14

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models

9 December 2024

15

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

VEGF protects motor neurons against excitotoxicity by upregulation of GluR2.

9 December 2024

16

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Increased oxidative damage to DNA in ALS patients

9 December 2024

17

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis.

9 December 2024

18

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Identification and characterization of cholest-4-en-3-one, oxime (TRO19622), a novel drug candidate for amyotrophic lateral sclerosis

9 December 2024

19

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial enzyme activity in amyotrophic lateral sclerosis: implications for the role of mitochondria in neuronal cell death

9 December 2024

20

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

9 December 2024

21

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis

9 December 2024

22

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Minocycline for patients with ALS

9 December 2024

23

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells

9 December 2024

24

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Targets in ALS: designing multidrug therapies.

9 December 2024

25

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Motor neurons are selectively vulnerable to AMPA/kainate receptor-mediated injury in vitro

9 December 2024

26

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidants

9 December 2024

27

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Dexpramipexole, the R(+) enantiomer of pramipexole, for the potential treatment of amyotrophic lateral sclerosis

9 December 2024

28

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases

9 December 2024

29

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)

9 December 2024

30

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Conversion of Bcl-2 to a Bax-like Death Effector by Caspases

9 December 2024

31

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutation of FIG4 causes neurodegeneration in the pale tremor mouse and patients with CMT4J

9 December 2024

32

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS).

9 December 2024

33

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Oligomerization of mutant SOD1 in mitochondria of motoneuronal cells drives mitochondrial damage and cell toxicity

9 December 2024

34

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Amyotrophic lateral sclerosis: from current developments in the laboratory to clinical implications

9 December 2024

35

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Apaf1 mediates apoptosis and mitochondrial damage induced by mutant human SOD1s typical of familial amyotrophic lateral sclerosis

9 December 2024

36

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Apoptosome inactivation rescues proneural and neural cells from neurodegeneration.

9 December 2024

37

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Bcl2a1 serves as a switch in death of motor neurons in amyotrophic lateral sclerosis

9 December 2024

38

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Manganese porphyrin given at symptom onset markedly extends survival of ALS mice

9 December 2024

39

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial respiratory chain dysfunction in muscle from patients with amyotrophic lateral sclerosis.

9 December 2024

40

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Amyotrophic lateral sclerosis: oxidative energy metabolism and calcium homeostasis in peripheral blood lymphocytes

9 December 2024

41

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The "dying-back" phenomenon of motor neurons in ALS.

9 December 2024

42

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis

9 December 2024

43

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice

9 December 2024

44

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

9 December 2024

45

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Role of axonal transport in neurodegenerative diseases

9 December 2024

46

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitophagy and Parkinson's disease: the PINK1-parkin link.

9 December 2024

47

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

9 December 2024

49

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models

9 December 2024

50

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondria in amyotrophic lateral sclerosis: a trigger and a target

9 December 2024

51

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegeneration

9 December 2024

52

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Antibodies to calcium channels from ALS patients passively transferred to mice selectively increase intracellular calcium and induce ultrastructural changes in motoneurons

9 December 2024

53

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation

9 December 2024

54

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Tolerance of high-dose (3,000 mg/day) coenzyme Q10 in ALS

9 December 2024

55

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Therapeutic efficacy of EGb761 (Gingko biloba extract) in a transgenic mouse model of amyotrophic lateral sclerosis

9 December 2024

56

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Evidence of Increased Oxidative Damage in Both Sporadic and Familial Amyotrophic Lateral Sclerosis

9 December 2024

57

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Calbindin D-28k and parvalbumin immunoreactivity in the frontal cortex in patients with frontal lobe dementia of non-Alzheimer type associated with amyotrophic lateral sclerosis.

9 December 2024

58

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials.

9 December 2024

59

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.

9 December 2024

60

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Activity of protein phosphatase calcineurin is decreased in sporadic and familial amyotrophic lateral sclerosispatients

9 December 2024

61

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Factors controlling the uptake of yeast copper/zinc superoxide dismutase into mitochondria.

9 December 2024

62

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Axonal degeneration in motor neuron disease

9 December 2024

63

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

9 December 2024

64

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis

9 December 2024

65

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice

9 December 2024

66

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Aberrant copper chemistry as a major mediator of oxidative stress in a human cellular model of amyotrophic lateral sclerosis

9 December 2024

67

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Inhibitory modulation of the mitochondrial permeability transition by minocycline

9 December 2024

68

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial.

9 December 2024

69

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS

9 December 2024

70

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis

9 December 2024

71

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Characterization of the AMPA-activated receptors present on motoneurons

9 December 2024

72

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Calcium dysregulation in amyotrophic lateral sclerosis

9 December 2024

73

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis

9 December 2024

74

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis

9 December 2024

75

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cloning and characterization of three novel genes, ALS2CR1, ALS2CR2, and ALS2CR3, in the juvenile amyotrophic lateral sclerosis (ALS2) critical region at chromosome 2q33-q34: candidate genes for ALS2.

9 December 2024

76

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Oxidative stress in skeletal muscle stimulates early expression of Rad in a mouse model of amyotrophic lateral sclerosis

9 December 2024

78

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Anterior horn cell degeneration and Bunina-type inclusions associated with dementia

9 December 2024

79

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Quantitative assessment of AMPA receptor mRNA in human spinal motor neurons isolated by laser capture microdissection

9 December 2024

80

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS

9 December 2024

81

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS).

9 December 2024

82

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The redox environment in the mitochondrial intermembrane space is maintained separately from the cytosol and matrix

9 December 2024

83

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions

9 December 2024

84

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

9 December 2024

85

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

9 December 2024

86

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations

9 December 2024

87

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The antioxidant N-acetylcysteine does not delay disease onset and death in a transgenic mouse model of amyotrophic lateral sclerosis

9 December 2024

88

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cu/Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of mitochondria and perturbs Ca2+ homeostasis in SOD1G93A mice

9 December 2024

89

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

9 December 2024

90

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Early activation of antioxidant mechanisms in muscle of mutant Cu/Zn-superoxide dismutase-linked amyotrophic lateral sclerosis mice

9 December 2024

91

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis

9 December 2024

92

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

9 December 2024

93

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Life span extension and reduced neuronal death after weekly intraventricular cyclosporin injections in the G93A transgenic mouse model of amyotrophic lateral sclerosis

9 December 2024

94

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Human spinal motoneurons express low relative abundance of GluR2 mRNA: an implication for excitotoxicity in ALS

9 December 2024

95

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

9 December 2024

96

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Intrathecal cyclosporin prolongs survival of late-stage ALS mice

9 December 2024

97

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Amyotrophic lateral sclerosis

9 December 2024

98

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Impairment of mitochondrial DNA repair enzymes against accumulation of 8-oxo-guanine in the spinal motor neurons of amyotrophic lateral sclerosis.

9 December 2024

99

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cytochrome c association with the inner mitochondrial membrane is impaired in the CNS of G93A-SOD1 mice.

9 December 2024

100

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

An ALS mouse model with a permeable blood-brain barrier benefits from systemic cyclosporine A treatment.

9 December 2024

101

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

9 December 2024

102

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies.

9 December 2024

103

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Antioxidant properties of minocycline: neuroprotection in an oxidative stress assay and direct radical-scavenging activity

9 December 2024

104

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis

9 December 2024

105

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial membrane permeabilization in cell death

9 December 2024

106

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

ALS-linked Cu/Zn-SOD mutation increases vulnerability of motor neurons to excitotoxicity by a mechanism involving increased oxidative stress and perturbed calcium homeostasis

9 December 2024

107

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

9 December 2024

108

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Minocycline for patients with ALS.

9 December 2024

109

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutant SOD1 and mitochondrial damage alter expression and splicing of genes controlling neuritogenesis in models of neurodegeneration.

9 December 2024

110

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.

9 December 2024

111

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model

9 December 2024

112

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Conversion of Bcl-2 from protector to killer by interaction with nuclear orphan receptor Nur77/TR3

9 December 2024

113

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Endogenous calcium buffering in motoneurones of the nucleus hypoglossus from mouse

9 December 2024

114

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids

9 December 2024

115

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

9 December 2024

116

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Increased mitochondrial antioxidative activity or decreased oxygen free radical propagation prevent mutant SOD1-mediated motor neuron cell death and increase amyotrophic lateral sclerosis-like transgenic mouse survival

9 December 2024

117

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis

9 December 2024

118

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Dynamic NAD(P)H post-synaptic autofluorescence signals for the assessment of mitochondrial function in a neurodegenerative disease: monitoring the primary motor cortex of G93A mice, an amyotrophic lateral sclerosis model.

9 December 2024

119

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The development of mitochondrial medicine

9 December 2024

120

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cortical selective vulnerability in motor neuron disease: a morphometric study.

9 December 2024

121

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

9 December 2024

122

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Oxidative stress and antioxidant enzyme upregulation in SOD1-G93A mouse skeletal muscle.

9 December 2024

123

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Olesoxime, a cholesterol-like neuroprotectant for the potential treatment of amyotrophic lateral sclerosis

9 December 2024

124

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice

9 December 2024

125

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Motor neuron degeneration in amyotrophic lateral sclerosis mutant superoxide dismutase-1 transgenic mice: mechanisms of mitochondriopathy and cell death.

9 December 2024

126

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Neuronal death in amyotrophic lateral sclerosis is apoptosis: possible contribution of a programmed cell death mechanism

9 December 2024

127

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

PINK1 stabilized by mitochondrial depolarization recruits Parkin to damaged mitochondria and activates latent Parkin for mitophagy

9 December 2024

128

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice

9 December 2024

129

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

9 December 2024

130

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

A disulfide relay system in the intermembrane space of mitochondria that mediates protein import

9 December 2024

131

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Optimal methods to characterize the G93A mouse model of ALS.

9 December 2024

132

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase

9 December 2024

133

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Induction of parkinsonism-related proteins in the spinal motor neurons of transgenic mouse carrying a mutant SOD1 gene

9 December 2024

134

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Increased autophagy in transgenic mice with a G93A mutant SOD1 gene

9 December 2024

135

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Early decrease of mitochondrial DNA repair enzymes in spinal motor neurons of presymptomatic transgenic mice carrying a mutant SOD1 gene

9 December 2024

136

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Hepatic ultrastructural changes and liver dysfunction in amyotrophic lateral sclerosis

9 December 2024

137

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Neuroprotective effect of Nrf2/ARE activators, CDDO ethylamide and CDDO trifluoroethylamide, in a mouse model of amyotrophic lateral sclerosis

9 December 2024

138

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The Psi(m) depolarization that accompanies mitochondrial Ca2+ uptake is greater in mutant SOD1 than in wild-type mouse motor terminals.

9 December 2024

139

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis

9 December 2024

140

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial protein oxidation in yeast mutants lacking manganese-(MnSOD) or copper- and zinc-containing superoxide dismutase (CuZnSOD): evidence that MnSOD and CuZnSOD have both unique and overlapping functions in protecting mitochondrial proteins

9 December 2024

141

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

9 December 2024

142

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Axonal swellings in the corticospinal tracts in amyotrophic lateral sclerosis

9 December 2024

143

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Calcium dynamics and buffering in motoneurones of the mouse spinal cord

9 December 2024

144

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria

9 December 2024

145

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

9 December 2024

146

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2.

9 December 2024

147

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The lipophilic metal chelators DP-109 and DP-460 are neuroprotective in a transgenic mouse model of amyotrophic lateral sclerosis

9 December 2024

148

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis

9 December 2024

149

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells

9 December 2024

150

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Cell culture models to investigate the selective vulnerability of motoneuronal mitochondria to familial ALS-linked G93ASOD1.

9 December 2024

151

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1.

9 December 2024

152

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis

9 December 2024

153

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

9 December 2024

154

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis

9 December 2024

155

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis

9 December 2024

156

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Rare missense variants of neuronal nicotinic acetylcholine receptor altering receptor function are associated with sporadic amyotrophic lateral sclerosis

9 December 2024

157

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial alterations in dorsal root ganglion cells in sporadic amyotrophic lateral sclerosis

9 December 2024

158

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Parvalbumin and calbindin D-28k immunoreactivity in transgenic mice with a G93A mutant SOD1 gene

9 December 2024

159

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial protein import: from proteomics to functional mechanisms

9 December 2024

160

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.

9 December 2024

161

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Studies on cellular free radical protection mechanisms in the anterior horn from patients with amyotrophic lateral sclerosis

9 December 2024

162

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Non-NMDA receptors in motor neuron disease (MND): a quantitative autoradiographic study in spinal cord and motor cortex using [3H]CNQX and [3H]kainate

9 December 2024

163

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation

9 December 2024

164

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Selective formation of certain advanced glycation end products in spinal cord astrocytes of humans and mice with superoxide dismutase-1 mutation

9 December 2024

165

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Ultrastructural evidence for altered calcium in motor nerve terminals in amyotropic lateral sclerosis

9 December 2024

166

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

9 December 2024

167

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

9 December 2024

168

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology

9 December 2024

169

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS.

9 December 2024

170

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

9 December 2024

171

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein

9 December 2024

172

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondria in sporadic amyotrophic lateral sclerosis

9 December 2024

173

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis

9 December 2024

174

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Chronic elevation of extracellular glutamate due to transport blockade is innocuous for spinal motoneurons in vivo

9 December 2024

175

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

9 December 2024

176

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

GluR2-dependent properties of AMPA receptors determine the selective vulnerability of motor neurons to excitotoxicity.

9 December 2024

177

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Ca(2+)-permeable AMPA receptors and selective vulnerability of motor neurons

9 December 2024

178

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

9 December 2024

179

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.

9 December 2024

180

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease

9 December 2024

181

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis

9 December 2024

182

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.

9 December 2024

183

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis

9 December 2024

184

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Proapoptotic BAX and BAK: a requisite gateway to mitochondrial dysfunction and death

9 December 2024

185

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Oxidative damage and mutation to mitochondrial DNA and age-dependent decline of mitochondrial respiratory function

9 December 2024

186

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial fusion and fission in cell life and death

9 December 2024

188

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

9 December 2024

189

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis

9 December 2024

190

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice

9 December 2024

191

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Focal cytochrome c oxidase deficiency in various neuromuscular diseases

9 December 2024

192

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

9 December 2024

193

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.

9 December 2024

194

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

Hyperactive intracellular calcium signaling associated with localized mitochondrial defects in skeletal muscle of an animal model of amyotrophic lateral sclerosis

9 December 2024

195

1 reference

https://api.crossref.org/works/10.1016/J.PNEUROBIO.2011.06.003

9 December 2024

Identifiers

1 reference

25 August 2017

1 reference