Entity Details

Primary name HOIL1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ9BYM8
EntryNameHOIL1_HUMAN
FullNameRanBP-type and C3HC4-type zinc finger-containing protein 1
TaxID9606
Evidenceevidence at protein level
Length510
SequenceStatuscomplete
DateCreated2001-09-26
DateModified2021-06-02

Ontological Relatives

GenesRBCK1

GO terms

Show/Hide Table
GOName
GO:0000151 ubiquitin ligase complex
GO:0000209 protein polyubiquitination
GO:0003713 transcription coactivator activity
GO:0004842 ubiquitin-protein transferase activity
GO:0005829 cytosol
GO:0007249 I-kappaB kinase/NF-kappaB signaling
GO:0010803 regulation of tumor necrosis factor-mediated signaling pathway
GO:0016032 viral process
GO:0032088 negative regulation of NF-kappaB transcription factor activity
GO:0042802 identical protein binding
GO:0043123 positive regulation of I-kappaB kinase/NF-kappaB signaling
GO:0043130 ubiquitin binding
GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process
GO:0046872 metal ion binding
GO:0050852 T cell receptor signaling pathway
GO:0051092 positive regulation of NF-kappaB transcription factor activity
GO:0051220 cytoplasmic sequestering of protein
GO:0060546 negative regulation of necroptotic process
GO:0071797 LUBAC complex
GO:0097039 protein linear polyubiquitination
GO:0140311 protein sequestering activity
GO:1901224 positive regulation of NIK/NF-kappaB signaling
GO:2001238 positive regulation of extrinsic apoptotic signaling pathway

Subcellular Location

Show/Hide Table

Domains

Show/Hide Table
DomainNameCategoryType
IPR000626 Ubiquitin-like domainDomainDomain
IPR001841 Zinc finger, RING-typeDomainDomain
IPR001876 Zinc finger, RanBP2-typeDomainDomain
IPR002867 IBR domainDomainDomain
IPR013083 Zinc finger, RING/FYVE/PHD-typeFamilyHomologous superfamily
IPR017907 Zinc finger, RING-type, conserved siteSiteConserved site
IPR026261 RanBP-type and C3HC4-type zinc finger-containing protein 1/SHARPINFamilyFamily
IPR027370 RING-type zinc-finger, LisH dimerisation motifDomainDomain
IPR029071 Ubiquitin-like domain superfamilyFamilyHomologous superfamily
IPR036443 Zinc finger, RanBP2-type superfamilyFamilyHomologous superfamily
IPR044066 TRIAD supradomainDomainDomain

Diseases

Show/Hide Table
Disease IDSourceNameDescription
615895 OMIMPolyglucosan body myopathy 1 with or without immunodeficiency (PGBM1)A disease characterized by polyglucosan storage myopathy associated with early-onset progressive muscle weakness and progressive dilated cardiomyopathy, which may necessitate cardiac transplant in severe cases. Some patients present with severe immunodeficiency, invasive bacterial infections and chronic autoinflammation. The disease is caused by variants affecting the gene represented in this entry.

Interactions

94 interactions

InteractorPartnerSourcesPublicationsLink
HOIL1_HUMANRNF31_HUMANBioGRID, DIP, HPRD, IntAct, MINT17006537 17069764 20005846 21455180 21455181 22430200 22791023 23032187 23708998 24141947 24726327 26264872 26525107 26577923 26670046 27307491 27545878 28514442 28978479 29892012 29997244 30561431 32019898 32122970 32814053 33329596 details
HOIL1_HUMANUB2G1_HUMANBioGRID, IntAct19549727 details
HOIL1_HUMANUB2L3_HUMANBioGRID, IntAct17006537 18303026 19549727 29643511 32296183 details
HOIL1_HUMANUBE2N_HUMANBioGRID, IntAct19549727 25260751 details
HOIL1_HUMANUB2L6_HUMANBioGRID, IntAct19549727 25416956 32296183 details
HOIL1_HUMANUBE2U_HUMANBioGRID, IntAct19549727 details
HOIL1_HUMANUBE2S_HUMANBioGRID, IntAct19549727 details
HOIL1_HUMANUB2D4_HUMANBioGRID, IntAct19549727 details
HOIL1_HUMANRO52_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANUHRF2_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANRN181_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANMKRN2_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANPCGF2_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANTRIML_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANTRI65_HUMANBioGRID, IntAct22493164 details
HOIL1_HUMANNEMO_HUMANBioGRID, DIP, IntAct, MINT19675569 20098747 21455180 21709223 21811235 23032186 23732909 23986494 27893701 28249776 30913450 details
HOIL1_HUMANSHRPN_HUMANBioGRID, DIP, IntAct, MINT21455180 21455181 23032186 24726327 26670046 28978479 30561431 30804189 31067453 33329596 details
HOIL1_HUMANTNAP3_HUMANBioGRID, MINT23032186 23105109 details
HOIL1_HUMANNAA10_HUMANBioGRID, IntAct25416956 details
HOIL1_HUMANTSSK3_HUMANBioGRID, IntAct25416956 32296183 details
HOIL1_HUMANNDUF3_HUMANBioGRID, IntAct25416956 details
HOIL1_HUMANSYCE1_HUMANBioGRID, IntAct25416956 details
HOIL1_HUMANHOIL1_HUMANBioGRID, IntAct16083853 18303026 25416956 30561431 details
HOIL1_HUMANPRKN_HUMANBioGRID, IntAct24722188 details
HOIL1_HUMANTTC23_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANGLCTK_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANENKD1_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANKANK2_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANMABP1_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANHXC8_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANLNX1_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANZN414_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANPSA1_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANCREB5_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANIL16_HUMANBioGRID, IntAct32296183 details
HOIL1_HUMANFKBPL_HUMANBioGRID, IntAct23912458 28514442 details
HOIL1_HUMANUBE2K_HUMANBioGRID, IntAct19549727 32814053 details
HOIL1_HUMANATX1_HUMANIntAct32814053 details
HOIL1_HUMANTADBP_HUMANIntAct32814053 details
HOIL1_HUMANUBC_HUMANBioGRID17006537 18303026 19796170 20005846 22139374 23986494 28190767 29694895 details
HOIL1_HUMANKPCB_HUMANBioGRID17069764 17121852 18303026 23042805 details
HOIL1_HUMANBACH1_HUMANBioGRID, HPRD17682061 details
HOIL1_HUMANUB2D3_HUMANBioGRID12629548 17006537 17682061 21292167 details
HOIL1_HUMANIREB2_HUMANBioGRID12629548 17822790 details
HOIL1_HUMANTRAF1_HUMANBioGRID21903422 25996949 27893701 details
HOIL1_HUMANTRI25_HUMANBioGRID21292167 details
HOIL1_HUMANNEDD4_HUMANBioGRID19953087 details
HOIL1_HUMANPSMD4_HUMANBioGRID19796170 22517668 details
HOIL1_HUMANESR1_HUMANBioGRID23042805 23912458 details
HOIL1_HUMANA4_HUMANBioGRID21832049 details
HOIL1_HUMANNR1I2_HUMANBioGRID23160820 details
HOIL1_HUMANUCHL5_HUMANBioGRID23105109 details
HOIL1_HUMANUBP2_HUMANBioGRID23105109 details
HOIL1_HUMANUBP7_HUMANBioGRID23105109 details
HOIL1_HUMANUBP15_HUMANBioGRID23105109 details
HOIL1_HUMANUBP21_HUMANBioGRID23105109 details
HOIL1_HUMANUBP32_HUMANBioGRID23105109 details
HOIL1_HUMANUBP49_HUMANBioGRID23105109 details
HOIL1_HUMANPAN2_HUMANBioGRID23105109 details
HOIL1_HUMANOTUB2_HUMANBioGRID23105109 details
HOIL1_HUMANOTU6A_HUMANBioGRID23105109 details
HOIL1_HUMANARNT2_HUMANBioGRID24722188 details
HOIL1_HUMANKPCA_HUMANBioGRID25118570 details
HOIL1_HUMANUB2D1_HUMANBioGRID17006537 details
HOIL1_HUMANUB2D2_HUMANBioGRID17006537 29643511 details
HOIL1_HUMANM3K1_HUMANBioGRID25260751 details
HOIL1_HUMANMALT1_HUMANBioGRID26525107 33329596 details
HOIL1_HUMANSMUF1_HUMANBioGRID20804422 details
HOIL1_HUMANHXA1_HUMANBioGRID27382069 details
HOIL1_HUMANHGS_HUMANBioGRID26871637 details
HOIL1_HUMANP53_HUMANBioGRID30874541 details
HOIL1_HUMANSOCS6_HUMANHPRD16643902 details
HOIL1_HUMANTNFA_HUMANBioGRID, DIP, InnateDB, MINT20005846 23032186 23104095 23955153 25911380 26670046 27307491 27545878 30420664 31898971 details
HOIL1_HUMANNOD2_HUMANBioGRID, IntAct22607974 23818254 26670046 details
HOIL1_HUMANDDX58_HUMANBioGRID21292167 32513696 details
HOIL1_HUMANTAB2_HUMANBioGRID17449468 details
HOIL1_HUMANTAB3_HUMANBioGRID17449468 details
HOIL1_HUMANM3K7_HUMANBioGRID17449468 details
HOIL1_HUMANTRAF6_HUMANBioGRID17449468 details
HOIL1_HUMANRIPK1_HUMANBioGRID17449468 29955050 details
HOIL1_HUMANNR2C2_HUMANBioGRID17449468 details
HOIL1_HUMANPML_HUMANBioGRID15833741 details
HOIL1_HUMANOTUL_HUMANBioGRID23708998 24726323 24726327 26670046 28919039 31504727 details
HOIL1_HUMANHS90A_HUMANBioGRID23912458 details
HOIL1_HUMANPDLI7_HUMANBioGRID27903798 details
HOIL1_HUMANTLR3_HUMANBioGRID27810922 details
HOIL1_HUMANTNR1A_HUMANBioGRID27552911 30420664 details
HOIL1_HUMANCETN3_HUMANBioGRID30251372 details
HOIL1_HUMANTNR1B_HUMANBioGRID29378181 details
HOIL1_HUMANHD_HUMANBioGRID30886048 details
HOIL1_HUMANCASP1_HUMANBioGRID32122970 details
HOIL1_HUMANCAR11_HUMANBioGRID33329596 details
HOIL1_HUMANBCL10_HUMANBioGRID33329596 details
HOIL1_HUMANCBP_HUMANHPRD15833741 details