ARHGAP26
Rho GTPase-activating protein 26
Also known as: GRAF, KIAA0621, OPHN1L, OPHN1L1, RHG26_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- Q9UNA1
- Gene
- ARHGAP26
- Ensembl
- ENSG00000145819
- Chromosome
- 5
- Canonical length
- 814 aa
- Protein class
- Cancer-related genes, Disease related genes, Human disease related genes, Predicted intracellular proteins, Predicted membrane proteins
- Subcellular location
- Cytosol
OverviewNCBI Gene
Interaction of a cell with the extracellular matrix triggers integrin cell surface receptors to begin signaling cascades that regulate the organization of the actin-cytoskeleton. One of the proteins involved in these cascades is focal adhesion kinase. The protein encoded by this gene is a GTPase activating protein that binds to focal adhesion kinase and mediates the activity of the GTP binding proteins RhoA and Cdc42. Defects in this gene are a cause of juvenile myelomonocytic leukemia (JMML). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2017]
Canonical amino-acid sequenceUniProt
814 residues, UniProt reviewed canonical sequence.
>Q9UNA1|ARHGAP26
1 MGLPALEFSD CCLDSPHFRE TLKSHEAELD KTNKFIKELI KDGKSLISAL KNLSSAKRKF
61 ADSLNEFKFQ CIGDAETDDE MCIARSLQEF ATVLRNLEDE RIRMIENASE VLITPLEKFR
121 KEQIGAAKEA KKKYDKETEK YCGILEKHLN LSSKKKESQL QEADSQVDLV RQHFYEVSLE
181 YVFKVQEVQE RKMFEFVEPL LAFLQGLFTF YHHGYELAKD FGDFKTQLTI SIQNTRNRFE
241 GTRSEVESLM KKMKENPLEH KTISPYTMEG YLYVQEKRHF GTSWVKHYCT YQRDSKQITM
301 VPFDQKSGGK GGEDESVILK SCTRRKTDSI EKRFCFDVEA VDRPGVITMQ ALSEEDRRLW
361 MEAMDGREPV YNSNKDSQSE GTAQLDSIGF SIIRKCIHAV ETRGINEQGL YRIVGVNSRV
421 QKLLSVLMDP KTASETETDI CAEWEIKTIT SALKTYLRML PGPLMMYQFQ RSFIKAAKLE
481 NQESRVSEIH SLVHRLPEKN RQMLQLLMNH LANVANNHKQ NLMTVANLGV VFGPTLLRPQ
541 EETVAAIMDI KFQNIVIEIL IENHEKIFNT VPDMPLTNAQ LHLSRKKSSD SKPPSCSERP
601 LTLFHTVQST EKQEQRNSII NSSLESVSSN PNSILNSSSS LQPNMNSSDP DLAVVKPTRP
661 NSLPPNPSPT SPLSPSWPMF SAPSSPMPTS STSSDSSPVR SVAGFVWFSV AAVVLSLARS
721 SLHAVFSLLV NFVPCHPNLH LLFDRPEEAV HEDSSTPFRK AKALYACKAE HDSELSFTAG
781 TVFDNVHPSQ EPGWLEGTLN GKTGLIPENY VEFLLocalizationUniProt · AlphaFold · HPA
Whether an antibody against ARHGAP26 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Cell surface
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.39
- Highest tissue expression
- 34 nTPM
Expression across tissuesHPA
Tissue
- bone marrow: 34 nTPM
- lung: 20 nTPM
- placenta: 20 nTPM
- cerebellum: 17 nTPM
- cerebral cortex: 17 nTPM
- appendix: 17 nTPM
Single-cell type
- neutrophils: 7,866 nCPM
- monocytes: 1,724 nCPM
- salivary acinar cells: 1,276 nCPM
- pituicytes/fscs: 1,250 nCPM
- sertoli cells: 1,200 nCPM
- microglia: 1,048 nCPM
Immune cell
- neutrophil: 25 nTPM
- NK-cell: 9.9 nTPM
- basophil: 4 nTPM
- classical monocyte: 3.9 nTPM
- gdT-cell: 3.9 nTPM
- myeloid DC: 3 nTPM
Brain region
- cerebellum: 107 nTPM
- cerebral cortex: 101 nTPM
- amygdala: 88 nTPM
- hypothalamus: 88 nTPM
- white matter: 85 nTPM
- basal ganglia: 78 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about ARHGAP26.
Disease | AllUniProt
Conditions ARHGAP26 is implicated in, by any mechanism.
- Leukemia, juvenile myelomonocytic (JMML) MIM:607785
Disease | AutoantibodyPubMed
Conditions in which antibodies against ARHGAP26 are reported. Each links to that disease's full target list.
ReferencesPubMed · IEDB
Publications for ARHGAP26 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
7 publications
- Two new cases of anti-Ca (anti-ARHGAP26/GRAF) autoantibody-associated cerebellar ataxia.
2013 · J Neuroinflammation · RCR 1.2 · 36 citations - Anti-ARHGAP26 Autoantibodies Are Associated With Isolated Cognitive Impairment.
2018 · Front Neurol · RCR 0.7 · 16 citations - GTPase Regulator Associated with Focal Adhesion Kinase 1 (GRAF1) Immunoglobulin-Associated Ataxia and Neuropathy.
2020 · Mov Disord Clin Pract · RCR 0.7 · 12 citations - Case report: Anti-ARHGAP26 autoantibodies in atypical dementia with Lewy bodies.
2023 · Front Dement · RCR 0.6 · 5 citations - Rho GTPase-activating protein 10 (ARHGAP10/GRAF2) is a novel autoantibody target in patients with autoimmune encephalitis.
2022 · J Neurol · RCR 0.5 · 6 citations
Show 2 more
- Rho GTPase-activating protein 17 (ARHGAP17) as additional autoimmune target in ARHGAP26-IgG/anti-Ca autoantibody-associated autoimmune encephalitis.
2023 · J Neurol · RCR 0.2 · 1 citations - Cerebellar ataxia and depression associated with anti-RhoGTPase-activating protein 26 antibody: A case report.
2025 · J Neuroimmunol
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 0.28
- gnomAD pLI
- 1
- gnomAD missense Z
- 2.11
- DepMap mean gene effect
- 0.03
- DepMap dependency class
- selective
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
- actin cytoskeleton organization
- mitophagy
- regulation of small GTPase mediated signal transduction
- signal transduction
Molecular functions
Cellular components
Protein domainsUniProt · Pfam · InterPro
- Rho GTPase-activating protein domain
- SH3 domain
- Pleckstrin homology domain
- BAR domain
- Rho GTPase activation protein
- PH-like domain superfamily
- AH/BAR domain superfamily
- SH3-like domain superfamily
- GRAF, PH domain
- GRAF family
- PH domain
- RhoGAP domain
- Variant SH3 domain
- BAR domain of APPL family
- GRAF, SH3 domain
- GRAF, BAR domain
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of ARHGAP26 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads ARHGAP26 as an antibody target. Whether an autoantibody or antibody against ARHGAP26 could matter depends on whether native ARHGAP26 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
ARHGAP26 is annotated at the cell surface, where native ARHGAP26 is exposed to circulating antibodies and is a prime autoantibody target that could block, deplete, or overstimulate it.
Annotation status
The present source text does not explicitly label ARHGAP26 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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