AFG3L2 ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU. Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form. In the C-terminal section; belongs to the peptidase M41 family. Ubiquitous. Highly expressed in the cerebellar Purkinje cells. Note: This description may include information from UniProtKB.
Protein type: Cell development/differentiation; Chaperone; EC 3.4.24.-; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Protease
Chromosomal Location of human Ortholog: 18p11.21
Cellular Component:  m-AAA complex; mitochondrial inner membrane; mitochondrion
Molecular Function:  ATP binding; metalloendopeptidase activity; metallopeptidase activity; protein binding; unfolded protein binding; zinc ion binding
Biological Process:  axonogenesis; calcium import into the mitochondrion; mitochondrial calcium ion homeostasis; mitochondrial calcium ion transmembrane transport; muscle fiber development; myelination; nerve development; neuromuscular junction development; protein autoprocessing; protein processing; proteolysis; regulation of multicellular organism growth; righting reflex
Disease: Optic Atrophy 12; Spastic Ataxia 5, Autosomal Recessive; Spinocerebellar Ataxia 28
Reference #:  Q9Y4W6 (UniProtKB)
Alt. Names/Synonyms: AFG3 (ATPase family gene 3, yeast)-like 2; AFG3 ATPase family gene 3-like 2; AFG3 ATPase family gene 3-like 2 (yeast); AFG3 ATPase family member 3-like 2; AFG3 like AAA ATPase 2; AFG3 like matrix AAA peptidase subunit 2; AFG3-like protein 2; AFG32; AFG3L2; ATPase family gene 3, yeast; ATPase family gene 3-like 2; FLJ25993; OPA12; Paraplegin-like protein; SCA28; SPAX5; spinocerebellar ataxia 28
Gene Symbols: AFG3L2
Molecular weight: 88,584 Da
Basal Isoelectric point: 8.81  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  InnateDB