Property Summary

NCBI Gene PubMed Count 47
Grant Count 4
Funding $224,942.47
PubMed Score 36.89
PubTator Score 31.86

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (10)

Disease log2 FC p
Waldenstrons macroglobulinemia 1.003 0.003
Multiple myeloma 1.409 0.000
osteosarcoma -1.111 0.001
glioblastoma 1.200 0.007
juvenile dermatomyositis 1.217 0.000
tuberculosis 1.400 0.000
group 3 medulloblastoma 1.100 0.000
posterior fossa group A ependymoma 1.100 0.000
ovarian cancer 1.100 0.005
dermatomyositis 1.200 0.007

Synonym

Accession Q9BYM8 O95623 Q86SL2 Q96BS3 Q9BYM9
Symbols XAP3
XAP4
HOIL1
PBMEI
PGBM1
RBCK2
RNF54
HOIL-1
ZRANB4
C20orf18
UBCE7IP3

Gene

PANTHER Protein Class (2)

PDB

2CRC   2LGY   4DBG  

 GO Component (2)

Gene RIF (20)

PMID Text
26525107 Late in the NF-kappaB activation cycle HOIL1 cleavage transiently reduces linear ubiquitination, including of NEMO and RIP1, dampening NF-kappaB activation and preventing reactivation.
26008899 human HOIP is essential for the assembly and function of LUBAC, which includes HOIL-1, and for various processes governing inflammation and immunity in both hematopoietic and nonhematopoietic cells
23912458 findings support a role for RBCK1 in the regulation of FKBPL with important implications for estrogen receptor signaling, cell proliferation and response to endocrine therapy.
23798481 RBCK1 deficiency is a frequent cause of polyglucosan storage myopathy associated with progressive muscle weakness and cardiomyopathy.
23160820 Suggest that RBCK1 is important for the ubiquitination of PXR and may play a role in its proteasomal degradation.
23104095 Patients from two kindreds of a new fatal inherited disorder characterized by chronic autoinflammation, invasive bacterial infections and muscular amylopectinosis carried biallelic loss-of-expression and loss-of-function mutations in HOIL1.
23042805 Estrogen receptor-alpha, RBCK1, and protein kinase C beta 1 cooperate to regulate estrogen receptor-alpha gene expression.
22517668 The solution structure of the HOIL1 Ubl domain was solved using NMR spectroscopy to compare it with that of parkin to determine the structural elements responsible for S5a subunit of the 26S proteasome intermolecular interactions.
22139374 analysis of recognition of linear ubiquitin chains by the Npl4 zinc finger (NZF) domain of the HOIL-1L subunit of the linear ubiquitin chain assembly complex
20956555 Data report the identification of the related proteins Sipl1 (Shank-interacting protein-like 1) and Rbck1 (RBCC protein interacting with PKC1) as novel interaction partners of Eya1.
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AA Sequence

MDEKTKKAEEMALSLTRAVAGGDEQVAMKCAIWLAEQRVPLSVQLKPEVSPTQDIRLWVSVEDAQMHTVT      1 - 70
IWLTVRPDMTVASLKDMVFLDYGFPPVLQQWVIGQRLARDQETLHSHGVRQNGDSAYLYLLSARNTSLNP     71 - 140
QELQRERQLRMLEDLGFKDLTLQPRGPLEPGPPKPGVPQEPGRGQPDAVPEPPPVGWQCPGCTFINKPTR    141 - 210
PGCEMCCRARPEAYQVPASYQPDEEERARLAGEEEALRQYQQRKQQQQEGNYLQHVQLDQRSLVLNTEPA    211 - 280
ECPVCYSVLAPGEAVVLRECLHTFCRECLQGTIRNSQEAEVSCPFIDNTYSCSGKLLEREIKALLTPEDY    281 - 350
QRFLDLGISIAENRSAFSYHCKTPDCKGWCFFEDDVNEFTCPVCFHVNCLLCKAIHEQMNCKEYQEDLAL    351 - 420
RAQNDVAARQTTEMLKVMLQQGEAMRCPQCQIVVQKKDGCDWIRCTVCHTEICWVTKGPRWGPGGPGDTS    421 - 490
GGCRCRVNGIPCHPSCQNCH                                                      491 - 510
//

Text Mined References (50)

PMID Year Title
27523608 2016 The Deubiquitinase OTULIN Is an Essential Negative Regulator of Inflammation and Autoimmunity.
26525107 2015 The paracaspase MALT1 cleaves HOIL1 reducing linear ubiquitination by LUBAC to dampen lymphocyte NF-?B signalling.
26008899 2015 Human HOIP and LUBAC deficiency underlies autoinflammation, immunodeficiency, amylopectinosis, and lymphangiectasia.
25416956 2014 A proteome-scale map of the human interactome network.
25260751 2014 The MEKK1 PHD ubiquitinates TAB1 to activate MAPKs in response to cytokines.
24726323 2014 Molecular basis and regulation of OTULIN-LUBAC interaction.
23912458 2014 Identification of RBCK1 as a novel regulator of FKBPL: implications for tumor growth and response to tamoxifen.
23798481 2013 Polyglucosan body myopathy caused by defective ubiquitin ligase RBCK1.
23453807 2013 The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO.
23186163 2013 Toward a comprehensive characterization of a human cancer cell phosphoproteome.
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