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Target intelligence / Profile preview
Matrix AAA peptidase interacting protein 1 (MAIP1)
- Target
- MAIP1
- Molecular classification
- Other, Mitochondrial matrix protein
01
Overview
Matrix AAA peptidase interacting protein 1 (MAIP1) is a mitochondrial matrix-localized protein that facilitates calcium import through its role in sorting and maturing the SMDT1/EMRE protein, thereby ensuring proper mitochondrial calcium ion homeostasis. MAIP1 binds the transit peptide region of SMDT1/EMRE precursor, shielding this precursor from degradation by YME1L1 and allowing its maturation via mitochondrial processing peptidases (PMPCA and PMPCB). This protein is associated with rare neurological diseases, notably spastic ataxia 5 and spinocerebellar ataxia 28, but is not a common therapeutic target such as a receptor, enzyme, or transporter[1][5][6].
- Other names
- m-AAA protease-interacting protein 1, mitochondrialC2orf47DKFZp666A212FLJ22555Matrix AAA peptidase-interacting protein 1
02
Biological functions
Calcium import into the mitochondrionMitochondrial calcium ion homeostasisProtein insertion into mitochondrial membraneRibosome binding activityPromotion of SMDT1/EMRE maturation/protection against degradation[1][5][6]
03
Disease associations
Spastic ataxia 5Spinocerebellar ataxia 28[1]
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