(Q35540059)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

title

Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophy (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

main subject

spinal muscular atrophy

1 reference

1 reference

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Cindy Shin-Yi Lin

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

author name string

Michelle A Farrar

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Susanna B Park

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Heather M Johnston

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Desirée du Sart

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Hugh Bostock

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

Matthew C Kiernan

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

language of work or name

English

0 references

publication date

16 September 2011

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

volume

134

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

issue

Pt 11

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

page(s)

3185-3197

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy

12 February 2020

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Nerve excitability studies characterize Kv1.1 fast potassium channel dysfunction in patients with episodic ataxia type 1.

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Compound muscle action potential and motor function in children with spinal muscular atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Riluzole, neuroprotection and amyotrophic lateral sclerosis.

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Spinal muscular atrophy: molecular mechanisms

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

An analysis of disease severity based on SMN2 copy number in adults with spinal muscular atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

A positive modifier of spinal muscular atrophy in the SMN2 gene

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Phase II open label study of valproic acid in spinal muscular atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Motor axon excitability during Wallerian degeneration

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Natural history of denervation in SMA: relation to age, SMN2 copy number, and function

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Acute tetrodotoxin-induced neurotoxicity after ingestion of puffer fish

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

A Phase 1 Trial of Riluzole in Spinal Muscular Atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization.

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Muscle cramp in Machado-Joseph disease: altered motor axonal excitability properties and mexiletine treatment

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Quantitative analyses of SMN1 and SMN2 based on real-time lightCycler PCR: fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Quantitative analysis of survival motor neuron copies: identification of subtle SMN1 mutations in patients with spinal muscular atrophy, genotype-phenotype correlation, and implications for genetic counseling

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Threshold tracking techniques in the study of human peripheral nerve

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Correlation between severity and SMN protein level in spinal muscular atrophy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Identification of proximal spinal muscular atrophy carriers and patients by analysis of SMNT and SMNC gene copy number

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Identification and characterization of a spinal muscular atrophy-determining gene

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Molecular dissection of the myelinated axon

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

The strength-duration relationship for excitation of myelinated nerve: computed dependence on membrane parameters

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Function and distribution of three types of rectifying channel in rat spinal root myelinated axons.

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Changes in excitability of human motor axons underlying post-ischaemic fasciculations: evidence for two stable states

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Peripheral Nerve Excitability Measures at Different Target Levels: The Effects of Aging and Diabetic Neuropathy

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

In vivo assessment of HCN channel current (I(h)) in human motor axons

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Changes in excitability properties associated with axonal regeneration in human neuropathy and mouse Wallerian degeneration

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Axonal excitability properties in amyotrophic lateral sclerosis

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Altered nerve excitability properties in established diabetic neuropathy

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Increased persistent Na(+) current and its effect on excitability in motoneurones cultured from mutant SOD1 mice.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Nerve excitability properties in Charcot-Marie-Tooth disease type 1A.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Motor unit number estimation in infants and children with spinal muscular atrophy

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Molecular analysis of spinal muscular atrophy and modification of the phenotype by SMN2.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Latent addition in motor and sensory fibres of human peripheral nerve

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Differences in the recovery of excitability in sensory and motor axons of human median nerve

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Strength-duration properties of human peripheral nerve

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Action potentials and membrane currents in the human node of Ranvier

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Time- and frequency-dependent effects of potassium channel blockers on large and medium diameter optic tract axons

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Changes in Na+ channel expression and nodal persistent Na+ currents associated with peripheral nerve regeneration in mice

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3212713

Multiple measures of axonal excitability: a new approach in clinical testing

15 November 2018

1 reference

12 December 2020

Riluzole inhibits the persistent sodium current in mammalian CNS neurons

1 reference

12 December 2020

Effects of membrane polarization and ischaemia on the excitability properties of human motor axons

1 reference

12 December 2020

Effects of temperature on the excitability properties of human motor axons

1 reference

12 December 2020

Depletion and sizes of motor units in spinal muscular atrophy

1 reference

12 December 2020

Amyotrophic lateral sclerosis

1 reference

12 December 2020

Population screening and cascade testing for carriers of SMA

1 reference

12 December 2020

Counting motor units in chronic motor neuropathies

1 reference

12 December 2020

Intracellular recording from vertebrate myelinated axons: mechanism of the depolarizing afterpotential

1 reference

12 December 2020

Sodium and potassium channels in regenerating and developing mammalian myelinated nerves

1 reference

12 December 2020

Electromyographic findings in different forms of infantile and juvenile proximal spinal muscular atrophy

1 reference

12 December 2020

Peripheral nerve involvement in Werdnig-Hoffmann disease

1 reference

12 December 2020

International SMA consortium meeting. (26-28 June 1992, Bonn, Germany)

1 reference

12 December 2020

Axonal ion channel dysfunction in amyotrophic lateral sclerosis

1 reference

12 December 2020

Relationship between myelin sheath diameter and internodal length in axons of the anterior medullary velum of the adult rat

1 reference

12 December 2020

Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy

1 reference

12 December 2020

Outwardly rectifying deflections in threshold electrotonus due to K+ conductances

1 reference

12 December 2020

Pathophysiologic insights into motor axonal function in Kennedy disease

1 reference

12 December 2020

Plasticity of lower limb motor axons after cervical cord injury

1 reference

12 December 2020

Effect of maturation on nerve excitability in an experimental model of threshold electrotonus

1 reference

12 December 2020

Identifiers

DOI

10.1093/BRAIN/AWR229

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21926101%20AND%20SRC:MED&resulttype=core&format=json

12 February 2020

PubMed publication ID

21926101

1 reference