Proteins/UNIPROT:Q2TBA0

KLHL40

ProteinUNIPROT:Q2TBA0

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a key regulator of skeletal muscle development. The BCR(KLHL40) complex acts by mediating ubiquitination and degradation of TFDP1, thereby regulating the activity of the E2F:DP transcription factor complex (By similarity). Promotes stabilization of LMOD3 by acting as a negative regulator of LMOD3 ubiquitination; the molecular process by which it negatively regulates ubiquitination of LMOD3 is however unclear (By similarity)..

Function from UniProtKB (Q2TBA0), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

nemaline myopathy 8 has a germline basis in KLHL40DiseaseGenCC HGNC:30372|MONDO:0014138Inferred only
severe congenital nemaline myopathy has a germline basis in KLHL40DiseaseGenCC HGNC:30372|MONDO:0015735Inferred only

Statements

22 statements

22 statements · 2 reference databases · Subject: KLHL40 · Sources: gene ontology, Hetionet

Order

KLHL40 covaries with CARMIL2ProteinHetionet hetionet:covaries:131377-146206Inferred only
CDX1 covaries with KLHL40ProteinHetionet hetionet:covaries:1044-131377Inferred only
KLHL40 covaries with FBXL12ProteinHetionet hetionet:covaries:131377-54850Inferred only
GNL3L covaries with KLHL40ProteinHetionet hetionet:covaries:131377-54552Inferred only
KLHL40 covaries with GPR162ProteinHetionet hetionet:covaries:131377-27239Inferred only
IRS4 covaries with KLHL40ProteinHetionet hetionet:covaries:131377-8471Inferred only
KLHL40 covaries with LRRC41ProteinHetionet hetionet:covaries:10489-131377Inferred only
KLHL40 covaries with LYPLA2ProteinHetionet hetionet:covaries:11313-131377Inferred only
KLHL40 covaries with MYH7BProteinHetionet hetionet:covaries:131377-57644Inferred only
KLHL40 covaries with NAT14ProteinHetionet hetionet:covaries:131377-57106Inferred only
OR4Q3 covaries with KLHL40ProteinHetionet hetionet:covaries:131377-441669Inferred only
OR51G1 covaries with KLHL40ProteinHetionet hetionet:covaries:131377-79324Inferred only

Show the remaining 10

KLHL40 covaries with SPRED3ProteinHetionet hetionet:covaries:131377-399473Inferred only
KLHL40 covaries with STRCProteinHetionet hetionet:covaries:131377-161497Inferred only
KLHL40 covaries with WNT1ProteinHetionet hetionet:covaries:131377-7471Inferred only
KLHL40 covaries with ZNF200ProteinHetionet hetionet:covaries:131377-7752Inferred only
ZNF331 covaries with KLHL40ProteinHetionet hetionet:covaries:131377-55422Inferred only
ZNF502 covaries with KLHL40ProteinHetionet hetionet:covaries:131377-91392Inferred only
KLHL40 regulates negative regulation of proteasomal ubiquitin-dependent protein catabolic processBiological processgene ontology Q2TBA0|GO:0032435Inferred only
KLHL40 regulates negative regulation of protein ubiquitinationBiological processgene ontology Q2TBA0|GO:0031397Inferred only
KLHL40 regulates positive regulation of proteasomal ubiquitin-dependent protein catabolic processBiological processgene ontology Q2TBA0|GO:0032436Inferred only
KLHL40 regulates positive regulation of protein ubiquitinationBiological processgene ontology Q2TBA0|GO:0031398Inferred only

Other names

4 names

Resolves to: KLHL40

Also called: KBTBD5Kelch repeat and BTB domain-containing protein 5SarcosynapsinSRYP

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