WDR41

WDR41 (WD Repeat Domain 41) is a Protein Coding gene. Diseases associated with WDR41 include Frontotemporal Dementia And/Or Amyotrophic Lateral Sclerosis 1 and Amyotrophic Lateral Sclerosis 1.

Full Name

WD repeat domain 41

Function

Non-catalytic component of the C9orf72-SMCR8 complex, a complex that has guanine nucleotide exchange factor (GEF) activity and regulates autophagy (PubMed:27193190, PubMed:27103069, PubMed:27617292, PubMed:28195531).
The C9orf72-SMCR8 complex promotes the exchange of GDP to GTP, converting inactive GDP-bound RAB8A and RAB39B into their active GTP-bound form, thereby promoting autophagosome maturation (PubMed:27103069).
As part of the C9orf72-SMCR8 complex, stimulates RAB8A and RAB11A GTPase activity in vitro, however WDR42 is shown not be an essential complex component for this function (PubMed:32303654).
The C9orf72-SMCR8 complex also acts as a negative regulator of autophagy initiation by interacting with the ULK1/ATG1 kinase complex and inhibiting its protein kinase activity (PubMed:27103069, PubMed:27617292).

Biological Process

Biological Process autophagy Source:UniProtKB-KW
Biological Process negative regulation of exocytosis Source:ComplexPortal1 Publication
Biological Process negative regulation of immune response Source:ComplexPortal1 Publication
Biological Process positive regulation of GTPase activity Source:UniProtKB1 Publication
Biological Process regulation of autophagy Source:UniProtKB1 Publication

Cellular Location

Cytoplasm