Lon protease homolog, mitochondrial

Gene:

LONP1 Enzyme: EC 3.4.21.53 This enzyme has known catalytic activity involved in reaction: Hydrolysis of proteins in presence of ATP. Disease CODAS syndrome: A rare syndrome characterized by the combination of cerebral, ocular, dental, auricular, and skeletal features. These include developmental delay, craniofacial anomalies, cataracts, ptosis, median nasal groove, delayed tooth eruption, hearing loss, short stature, delayed epiphyseal ossification, metaphyseal hip dysplasia, and vertebral coronal clefts... [click for more]

Organism:

Homo sapiens (Human)

Synonyms:

PRSS15

Uniprot:

P36776go to UniProt

Biological function:

ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix (PubMed:12198491, PubMed:15870080, PubMed:17579211, PubMed:37327776, PubMed:8248235). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and ... [show more]go to UniProt

Representative structure for UniProt P36776

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PDB 7oxo chain B (observed residues)
PDB 7oxo chain B (observed residues)
PDB 7oxo chain B (observed residues)
PDB 7oxo chain B (observed residues)

PDB chain shown: 7oxo B go to PDBe

UniProt residues 1 - 959

Coverage: 81%

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27
Structures

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10
Ligands

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2
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63
Similarity

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48
Publications

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