ARHGAP26
Domain
The BAR domain is important to associate RAB8A (or RAB8B) and RAB10 to endosomal membrane to promote tubule extension. The BAR domain is also important to recruit WDR44 to endosomal tubules.
Function
GTPase-activating protein for RHOA and CDC42. Facilitates mitochondrial quality control by promoting Parkin-mediated recruitment of autophagosomes to damaged mitochondria (PubMed:38081847). Negatively regulates the growth of human parainfluenza virus type 2 by inhibiting hPIV-2-mediated RHOA activation via interaction with two of its viral proteins P and V (PubMed:27512058).
Isoform 2
Associates with MICAL1 on the endosomal membrane to promote Rab8-Rab10-dependent tubule extension. After dissociation of MICAL1, recruits WDR44 which connects the endoplasmic reticulum (ER) with the endosomal tubule, thereby participating in the export of a subset of neosynthesized proteins.
Involvement in disease
Leukemia, juvenile myelomonocytic
JMML
An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages.
None
The gene represented in this entry is involved in disease pathogenesis. A chromosomal translocation t(5;11)(q31;q23) with KMT2A/MLL1 has been found in leukemic cells from JMML patients, also carrying inactivating mutations on the second allele (PubMed:10908648).
Post-translational modifications
Phosphorylated in a PINK1-dependent fashion promoting retrograde mitochondrial trafficking and clustering.
Cellular localization
- Isoform 2
- Endosome membrane
- Colocalized with RAB8A, RAB8B and RAB10 on endosomal tubules.
- Cytoplasm
- Cell junction
- Focal adhesion
- Cytoplasm
- Cytoskeleton
- Colocalizes with actin stress fibers and cortical actin structures.
Alternative names
GRAF, KIAA0621, OPHN1L, ARHGAP26, Rho GTPase-activating protein 26, GTPase regulator associated with focal adhesion kinase, Oligophrenin-1-like protein, Rho-type GTPase-activating protein 26, GRAF1