AFG3L2 (AFG3 like matrix AAA peptidase subunit 2)

2016-10-01  

Identity

HGNC
LOCATION
18p11.21
LOCUSID
ALIAS
OPA12,SCA28,SPAX5
FUSION GENES

Other Information

Locus ID:

NCBI: 10939
MIM: 604581
HGNC: 315
Ensembl: ENSG00000141385

Variants:

dbSNP: 10939
ClinVar: 10939
TCGA: ENSG00000141385
COSMIC: AFG3L2

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000141385ENST00000269143Q9Y4W6
ENSG00000141385ENST00000590811K7EP56

Expression (GTEx)

0
10
20
30
40
50
60
70
80
90

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
381578462024Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis.3
381578462024Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis.3
364471122023AFG3L2 Biallelic Mutation: Clinical Heterogeneity in Two Italian Patients.1
379177492023Autoregulatory control of mitochondrial glutathione homeostasis.9
364471122023AFG3L2 Biallelic Mutation: Clinical Heterogeneity in Two Italian Patients.1
379177492023Autoregulatory control of mitochondrial glutathione homeostasis.9
343333792021Expanding the phenotype of AFG3L2 mutations: Late-onset autosomal recessive spinocerebellar ataxia.5
343333792021Expanding the phenotype of AFG3L2 mutations: Late-onset autosomal recessive spinocerebellar ataxia.5
322198682020ATPase Domain AFG3L2 Mutations Alter OPA1 Processing and Cause Optic Neuropathy.20
322480512020Spastic ataxia with eye-of-the-tiger-like sign in 4 siblings due to novel compound heterozygous AFG3L2 mutation.3
326004592020A novel AFG3L2 mutation close to AAA domain leads to aberrant OMA1 and OPA1 processing in a family with optic atrophy.8
330750642020Inactivation of the mitochondrial protease Afg3l2 results in severely diminished respiratory chain activity and widespread defects in mitochondrial gene expression.7
322198682020ATPase Domain AFG3L2 Mutations Alter OPA1 Processing and Cause Optic Neuropathy.20
322480512020Spastic ataxia with eye-of-the-tiger-like sign in 4 siblings due to novel compound heterozygous AFG3L2 mutation.3
326004592020A novel AFG3L2 mutation close to AAA domain leads to aberrant OMA1 and OPA1 processing in a family with optic atrophy.8

Citation

Dessen P

AFG3L2 (AFG3 like matrix AAA peptidase subunit 2)

Atlas Genet Cytogenet Oncol Haematol. 2016-10-01

Online version: http://atlasgeneticsoncology.org/gene/56528/afg3l2-(afg3-like-matrix-aaa-peptidase-subunit-2)