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Protein ARHGAP26 PDB 1f7c.png
Available structures
PDB Ortholog search: PDBe RCSB
Aliases ARHGAP26, GRAF, GRAF1, OPHN1L, OPHN1L1, Rho GTPase activating protein 26
External IDs MGI: 1918552 HomoloGene: 36349 GeneCards: ARHGAP26
Gene location (Human)
Chromosome 5 (human)
Chr. Chromosome 5 (human)[1]
Chromosome 5 (human)
Genomic location for ARHGAP26
Genomic location for ARHGAP26
Band No data available Start 142,770,384 bp[1]
End 143,229,011 bp[1]
RNA expression pattern
PBB GE ARHGAP26 205069 s at fs.png

PBB GE ARHGAP26 205068 s at fs.png

PBB GE ARHGAP26 215955 x at fs.png
More reference expression data
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 5: 142.77 – 143.23 Mb Chr 5: 38.6 – 39.38 Mb
PubMed search [3] [4]
View/Edit Human View/Edit Mouse

Rho GTPase activating protein 26 (ARHGAP26) also known as GTPase Regulator Associated with Focal Adhesion Kinase (GRAF) is a protein that in humans is encoded by the ARHGAP26 gene.[5][6][7]


GRAF1 is a multidomain protein that is necessary for the CLIC/GEEC endocytic pathway.[8] By virtue of an N-terminal BAR domain, GRAF1 sculpts the endocytic membranes of this pathway into 40 nm diameter tubules and vesicles that allow uptake of extracellular fluid, GPI-linked proteins and certain bacterial exotoxins into cells. The role of dynamin in the CLIC/GEEC pathway is controversial, but GRAF1 interacts strongly with this protein and acute inhibition of dynamin action abrogates CLIC/GEEC endocytosis. There are several members of the GRAF family of proteins, including GRAF2, GRAF3, and oligophrenin, all of which likely playing similar roles during clathrin-independent endocytic events. Mutations of both GRAF1 and oligophrenin are strongly implicated in causing human disease (leukaemia and mental retardation, respectively). Recently, autoantibodies to ARHGAP26 have been implicated in autoimmune cerebellar ataxia.[9][10][11]


ARHGAP26 has been shown to interact with PKN3.[12]


  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000145819 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000036452 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:". 
  4. ^ "Mouse PubMed Reference:". 
  5. ^ "Entrez Gene: ARHGAP26 Rho GTPase activating protein 26". 
  6. ^ Hildebrand JD, Taylor JM, Parsons JT (June 1996). "An SH3 domain-containing GTPase-activating protein for Rho and Cdc42 associates with focal adhesion kinase". Mol. Cell. Biol. 16 (6): 3169–78. PMC 231310Freely accessible. PMID 8649427. 
  7. ^ Taylor JM, Macklem MM, Parsons JT (January 1999). "Cytoskeletal changes induced by GRAF, the GTPase regulator associated with focal adhesion kinase, are mediated by Rho". J. Cell. Sci. 112 (2): 231–42. PMID 9858476. 
  8. ^ Lundmark R, Doherty GJ, Howes MT, Cortese K, Vallis Y, Parton RG, McMahon HT (November 2008). "The GTPase-activating protein GRAF1 regulates the CLIC/GEEC endocytic pathway". Curr. Biol. 18 (22): 1802–8. PMC 2726289Freely accessible. PMID 19036340. doi:10.1016/j.cub.2008.10.044. 
  9. ^ Jarius S, Wandinger KP, Horn S, Heuer H, Wildemann B (Mar 2012). "A new Purkinje cell antibody (anti-Ca) associated with subacute cerebellar ataxia: immunological characterization". J Neuroinflammation. 7 (1): 21. PMC 2848133Freely accessible. PMID 20226058. doi:10.1186/1742-2094-7-21.  Check date values in: |year= / |date= mismatch (help)
  10. ^ Jarius S, Martínez-García P, Hernandez AL, Brase JC, Borowski K, Regula JU, Meinck HM, Stöcker W, Wildemann B, Wandinger KP (Jan 2013). "Two new cases of anti-Ca (anti-ARHGAP26/GRAF) autoantibody-associated cerebellar ataxia". J Neuroinflammation. 10 (1): 7. PMC 3549891Freely accessible. PMID 23320754. doi:10.1186/1742-2094-10-7. 
  11. ^ Doss S, Nümann A, Ziegler A, Siebert E, Borowski K, Stöcker W, Prüss H, Wildemann B, Endres M, Jarius S (15 Feb 2014). "Anti-Ca/anti-ARHGAP26 antibodies associated with cerebellar atrophy and cognitive decline". J. Neuroimmunol. 267 (1–2): 102–4. PMID 24439423. doi:10.1016/j.jneuroim.2013.10.010. 
  12. ^ Shibata H, Oishi K, Yamagiwa A, Matsumoto M, Mukai H, Ono Y (2001). "PKNbeta interacts with the SH3 domains of Graf and a novel Graf related protein, Graf2, which are GTPase activating proteins for Rho family". J. Biochem. 130 (1): 23–31. PMID 11432776. doi:10.1093/oxfordjournals.jbchem.a002958. 

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