KLHL40 (kelch like family member 40)

2013-11-01  

Identity

HGNC
LOCATION
3p22.1
LOCUSID
ALIAS
KBTBD5,NEM8,SRYP,SYRP

Other Information

Locus ID:

NCBI: 131377
MIM: 615340
HGNC: 30372
Ensembl: ENSG00000157119

Variants:

dbSNP: 131377
ClinVar: 131377
TCGA: ENSG00000157119
COSMIC: KLHL40

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000157119ENST00000287777Q2TBA0

Expression (GTEx)

0
50
100
150
200
250
300
350

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
353792542022Clinical and molecular analysis of four unrelated Chinese families with pathogenic KLHL40 variants causing nemaline myopathy 8.1
353792542022Clinical and molecular analysis of four unrelated Chinese families with pathogenic KLHL40 variants causing nemaline myopathy 8.1
339783232021A novel and recurrent KLHL40 pathogenic variants in a Chinese family of multiple affected neonates with nemaline myopathy 8.4
339783232021A novel and recurrent KLHL40 pathogenic variants in a Chinese family of multiple affected neonates with nemaline myopathy 8.4
323522462020The KLHL40 c.1516A>C is a Chinese-specific founder mutation causing nemaline myopathy 8: Report of six patients with pre- and postnatal phenotypes.9
323522462020The KLHL40 c.1516A>C is a Chinese-specific founder mutation causing nemaline myopathy 8: Report of six patients with pre- and postnatal phenotypes.9
277624392016Identification of KLHL40 mutations by targeted next-generation sequencing facilitated a prenatal diagnosis in a family with three consecutive affected fetuses with fetal akinesia deformation sequence.7
277624392016Identification of KLHL40 mutations by targeted next-generation sequencing facilitated a prenatal diagnosis in a family with three consecutive affected fetuses with fetal akinesia deformation sequence.7
249601632014KLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathy.63
249601632014KLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathy.63
237465492013Mutations in KLHL40 are a frequent cause of severe autosomal-recessive nemaline myopathy.95
237465492013Mutations in KLHL40 are a frequent cause of severe autosomal-recessive nemaline myopathy.95

Citation

Dessen P

KLHL40 (kelch like family member 40)

Atlas Genet Cytogenet Oncol Haematol. 2013-11-01

Online version: http://atlasgeneticsoncology.org/gene/53574/klhl40-(kelch-like-family-member-40)