Afg3l2 Gene Summary [Rat]

Predicted to enable ATP hydrolysis activity and metalloendopeptidase activity. Predicted to be involved in several processes, including calcium import into the mitochondrion; cellular response to glutathione; and proteolysis. Predicted to act upstream of or within several processes, including mitochondrion organization; nervous system development; and righting reflex. Predicted to be located in mitochondrion. Predicted to be part of m-AAA complex. Predicted to be active in mitochondrial inner membrane. Human ortholog(s) of this gene implicated in optic atrophy 12; spastic ataxia; spastic ataxia 5; and spinocerebellar ataxia type 28. Orthologous to human AFG3L2 (AFG3 like matrix AAA peptidase subunit 2). [provided by Alliance of Genome Resources, Apr 2025]

Details

Type
Protein Coding
Official Symbol
Afg3l2
Official Name
AFG3 like matrix AAA peptidase subunit 2 [Source:RGD Symbol;Acc:1305259]
Ensembl ID
ENSRNOG00000017965
Bio databases IDs NCBI: 307350 Ensembl: ENSRNOG00000017965
Aliases AFG3 like matrix AAA peptidase subunit 2
Synonyms 2310036I02RIK, AFG3-like AAA ATPase 2, AFG3 like matrix AAA peptidase subunit 2, Emv66, LOC102642902, OPA12, par, SCA28, SPAX5
Species
Rat, Rattus norvegicus
OrthologiesHumanMouse

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in rat Afg3l2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • peptidase
  • Peptidase family M41
  • protein binding
  • AAA+ lid domain
  • ATPase
  • AAA domain
  • AAA domain (dynein-related subfamily)
  • metallopeptidase
  • ATPase family associated with various cellular activities (AAA)
  • ATP-dependent metalloprotease FtsH
  • FtsH Extracellular
  • P-loop containing Nucleoside Triphosphate Hydrolases
  • ATPases associated with a variety of cellular activities
  • transporter
  • metalloendopeptidase

Pathways

Biological processes and signaling networks where the Afg3l2 gene in rat plays a role, providing insight into its function and relevance in health or disease.

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • optic atrophy type 12
  • autosomal recessive spastic ataxia type 5
  • spinocerebellar ataxia type 28
  • glycogen storage disease type III
  • optic atrophy
  • hereditary spastic ataxia
  • infection by HIV-1
regulated by
regulates
role in cell
  • degradation in
  • accumulation in
  • fusion
  • formation
  • organization
  • response by
  • development
  • turnover in
  • maturation in
  • stabilization in

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • pH resistant lipid raft fraction
  • Mitochondria
  • mitochondrial inner membrane
  • plasma

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the rat Afg3l2 gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • regulation of multicellular organism growth
  • righting reflex
  • cristae formation
  • protein catabolic process
  • mitochondrial fusion
  • muscle cell development
  • myelination
  • nerve development
  • neuromuscular junction development
  • axonogenesis
  • proteolysis
  • mitochondrial calcium ion homeostasis
  • membrane protein proteolysis
  • protein maturation
  • protein processing
  • protein autoprocessing
  • mitochondrial protein processing

Cellular Component

Where in the cell the gene product is active
  • m-AAA complex
  • mitochondrion
  • mitochondrial inner membrane

Molecular Function

What the gene product does at the molecular level
  • ATPase activity
  • ATP binding
  • zinc ion binding
  • protein binding
  • unfolded protein binding
  • ATP-dependent peptidase activity
  • metalloendopeptidase activity
  • metallopeptidase activity

Gene-Specific Assays for Results You Can Trust

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