FBXL4 (F-box and leucine rich repeat protein 4)

2019-04-01  

Identity

HGNC
LOCATION
6q16.1
LOCUSID
ALIAS
FBL4,FBL5,MTDPS13
FUSION GENES

Other Information

Locus ID:

NCBI: 26235
MIM: 605654
HGNC: 13601
Ensembl: ENSG00000112234

Variants:

dbSNP: 26235
ClinVar: 26235
TCGA: ENSG00000112234
COSMIC: FBXL4

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000112234ENST00000229971Q9UKA2
ENSG00000112234ENST00000369244Q9UKA2

Expression (GTEx)

0
5
10
15
20

Pathways

PathwaySourceExternal ID
Immune SystemREACTOMER-HSA-168256
Adaptive Immune SystemREACTOMER-HSA-1280218
Class I MHC mediated antigen processing & presentationREACTOMER-HSA-983169
Antigen processing: Ubiquitination & Proteasome degradationREACTOMER-HSA-983168

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
378762792024Excessive BNIP3- and BNIP3L-dependent mitophagy underlies the pathogenesis of FBXL4-mutated mitochondrial DNA depletion syndrome.0
378762792024Excessive BNIP3- and BNIP3L-dependent mitophagy underlies the pathogenesis of FBXL4-mutated mitochondrial DNA depletion syndrome.0
371023722023FBXL4 ubiquitin ligase deficiency promotes mitophagy by elevating NIX levels.9
371617842023FBXL4 suppresses mitophagy by restricting the accumulation of NIX and BNIP3 mitophagy receptors.14
371023722023FBXL4 ubiquitin ligase deficiency promotes mitophagy by elevating NIX levels.9
371617842023FBXL4 suppresses mitophagy by restricting the accumulation of NIX and BNIP3 mitophagy receptors.14
325252782020FBXL4 deficiency increases mitochondrial removal by autophagy.32
325595142020Novel homozygous mutation in the FBXL4 gene is associated with mitochondria DNA depletion syndrome-13.8
325252782020FBXL4 deficiency increases mitochondrial removal by autophagy.32
325595142020Novel homozygous mutation in the FBXL4 gene is associated with mitochondria DNA depletion syndrome-13.8
314425322019Characterization of the C584R variant in the mtDNA depletion syndrome gene FBXL4, reveals a novel role for FBXL4 as a regulator of mitochondrial fusion.12
314425322019Characterization of the C584R variant in the mtDNA depletion syndrome gene FBXL4, reveals a novel role for FBXL4 as a regulator of mitochondrial fusion.12
277434632017FBXL4 defects are common in patients with congenital lactic acidemia and encephalomyopathic mitochondrial DNA depletion syndrome.10
286986472017Identification of FBXL4 as a Metastasis Associated Gene in Prostate Cancer.10
289405062017Molecular and clinical spectra of FBXL4 deficiency.18

Citation

Dessen P

FBXL4 (F-box and leucine rich repeat protein 4)

Atlas Genet Cytogenet Oncol Haematol. 2019-04-01

Online version: http://atlasgeneticsoncology.org/gene/57816/fbxl4-(f-box-and-leucine-rich-repeat-protein-4)