Activity

Filter

Cancel
Date Panel Item Activity
5 actions
Motor Neurone Disease v2.10 HSPB1 Bryony Thompson Marked gene: HSPB1 as ready
Motor Neurone Disease v2.10 HSPB1 Bryony Thompson Gene: hspb1 has been classified as Amber List (Moderate Evidence).
Motor Neurone Disease v2.10 HSPB1 Bryony Thompson Classified gene: HSPB1 as Amber List (moderate evidence)
Motor Neurone Disease v2.10 HSPB1 Bryony Thompson Gene: hspb1 has been classified as Amber List (Moderate Evidence).
Motor Neurone Disease v2.9 HSPB1 Bryony Thompson gene: HSPB1 was added
gene: HSPB1 was added to Motor Neurone Disease. Sources: Literature
Mode of inheritance for gene: HSPB1 was set to BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Publications for gene: HSPB1 were set to 26768280; 27492805; 32334137
Phenotypes for gene: HSPB1 were set to amyotrophic lateral sclerosis MONDO:0004976
Review for gene: HSPB1 was set to AMBER
Added comment: HSPB1 variants are associated with distal hereditary motor neuropathy (dHMN).

HSPB1 variants reported in 3 sporadic cases (PMID 27492805, 32334137). Two missense more common than expected for AD disease in gnomAD and 1 frameshift variant at the end of the protein with functional analyses.

Homozygous HSPB1 variant is described in one consanguineous family (PMID 26768280) and segregates with ALS in 2 siblings. Heterozygous carriers are unaffected.
Sources: Literature