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AFG3L2 - AFG3 like matrix AAA peptidase subunit 2 (domestic cattle)

Gene
Symbol
Dates
  • Create:
    2016-09-14
  • Modify:
    2025-01-29

1 Names and Identifiers

1.1 Synonyms

  • mitochondrial inner membrane m-AAA protease component AFG3L2
  • AFG3 ATPase family gene 3-like 2
  • AFG3-like protein 2 (Paraplegin-like protein)

1.2 Other Identifiers

1.2.1 Ensembl ID

1.2.2 Bgee Gene ID

1.2.3 Enzyme Commission (EC) Number

1.2.4 VEuPathDB ID

3 Proteins

3.1 Protein Function

Catalytic component of the m-AAA protease, a protease that plays a key role in proteostasis of inner mitochondrial membrane proteins, and which is essential for axonal and neuron development. AFG3L2 possesses both ATPase and protease activities: the ATPase activity is required to unfold substrates, threading them into the internal proteolytic cavity for hydrolysis into small peptide fragments. The m-AAA protease carries out protein quality control in the inner membrane of the mitochondria by mediating degradation of mistranslated or misfolded polypeptides. The m-AAA protease complex also promotes the processing and maturation of mitochondrial proteins, such as MRPL32/bL32m, PINK1 and SP7. Mediates protein maturation of the mitochondrial ribosomal subunit MRPL32/bL32m by catalyzing the cleavage of the presequence of MRPL32/bL32m prior to assembly into the mitochondrial ribosome. Required for SPG7 maturation into its active mature form after SPG7 cleavage by mitochondrial-processing peptidase (MPP). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP). Acts as a regulator of calcium in neurons by mediating 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. Promotes the proteolytic degradation of GHITM upon hyperpolarization of mitochondria: progressive GHITM degradation leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2. Also acts as a regulator of mitochondrial glutathione homeostasis by mediating cleavage and degradation of SLC25A39. SLC25A39 cleavage is prevented when SLC25A39 binds iron-sulfur. Involved in the regulation of OMA1-dependent processing of OPA1. May act by mediating processing of OMA1 precursor, participating in OMA1 maturation.

3.2 Protein Targets

4 Interactions and Pathways

4.1 Pathways

5 Biochemical Reactions

6 Expression

7 Literature

7.1 Consolidated References

7.2 Gene-Chemical Co-Occurrences in Literature

7.3 Gene-Gene Co-Occurrences in Literature

7.4 Gene-Disease Co-Occurrences in Literature

8 Patents

8.1 Gene-Chemical Co-Occurrences in Patents

8.2 Gene-Gene Co-Occurrences in Patents

8.3 Gene-Disease Co-Occurrences in Patents

9 Information Sources

CONTENTS