STARD7
entrez
56910
canonical
uniprot
Q9NQZ5
canonical
ensembl
ENSG00000084090
resolver
ensp
ENSP00000338030
resolver
enst
ENST00000337288
resolver
genesymbol
STARD7
resolver
genesymbol_syn
GTT1
resolver
hgnc
HGNC:18063
resolver
uniprot
D3DXG9
resolver
uniprot
Q53T44
resolver
uniprot
Q6GU43
resolver
uniprot
Q969M6
resolver
uniprot_entry
STAR7_HUMAN
resolver
uniprot_sec
D3DXG9
resolver
uniprot_sec
Q53T44
resolver
uniprot_sec
Q6GU43
resolver
uniprot_sec
Q969M6
resolver
amino_acid_sequence
MLPRRLLAAWLAGTRGGGLLALLANQCRFVTGLRVRRAQQIAQLYGRLYSESSRRVLLGRLWRRLHGRPGHASALMAALAGVFVWDEERIQEEELQRSINEMKRLEEMSNMFQSSGVQHHPPEPKAQTEGNEDSEGKEQRWEMVMDKKHFKLWRRPITGTHLYQYRVFGTYTDVTPRQFFNVQLDTEYRKKWDALVIKLEVIERDVVSGSEVLHWVTHFPYPMYSRDYVYVRRYSVDQENNMMVLVSRAVEHPSVPESPEFVRVRSYESQMVIRPHKSFDENGFDYLLTYSDNPQTVFPRYCVSWMVSSGMPDFLEKLHMATLKAKNMEIKVKDYISAKPLEMSSEAKATSQSSERKNEGSCGPARIEYA
uniprot
proteins
function
FUNCTION: Mediates non-vesicular transfer of phosphatidylcholine to mitochondria, thereby maintaining mitochondrial membrane lipid composition, cristae organization, and respiratory function across diverse tissues. {ECO:0000269|PubMed:20042613, ECO:0000269|PubMed:28401922, ECO:0000269|PubMed:28821867, ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:32071354, ECO:0000269|PubMed:34416390}.; FUNCTION: [StAR-related lipid transfer protein 7, mitochondrial]: Transfers phosphatidylcholine (PC) from the endoplasmic reticulum to the mitochondrial outer membrane at ER-mitochondria contact sites, thereby maintaining mitochondrial PC content, respiratory complex stability and mitochondrial cristae morphology. {ECO:0000269|PubMed:28821867, ECO:0000269|PubMed:34416390}.; FUNCTION: [StAR-related lipid transfer protein 7, mature]: Transfers phosphatidylcholine (PC) between the mitochondrial outer and inner membranes, contributing to mitochondrial lipid homeostasis. The mature cytosolic and intermembrane space forms of STARD7 mediate non-vesicular PC transport by transiently associating with mitochondrial membranes. Required for normal cristae architecture and optimal activity of the respiratory chain (PubMed:29301859). Mediates intracellular distribution of coenzyme Q (ubiquinone), the intermembrane space form supports mitochondrial coenzyme Q biosynthesis and the cytosolic form transfers coenzyme Q to extramitochondrial membranes, enhancing antioxidant protection and limiting ferroptosis sensitivity (PubMed:36658222). {ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:36658222}.
uniprot
proteins
mass_dalton
43113
uniprot
proteins
mutagenesis
MUTAGEN 68; /note="R->D: No effect on proteolytic cleavage."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 72; /note="A->D: No effect on proteolytic cleavage."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 73; /note="S->D: No effect on proteolytic cleavage."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 74; /note="A->D: Proteolytic cleavage by PARL is slightly reduced."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 75; /note="L->D: Proteolytic cleavage by PARL is reduced."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 76; /note="M->D: Proteolytic cleavage by PARL is reduced."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 77; /note="A->D: Proteolytic cleavage by PARL is reduced."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 78; /note="A->D: Proteolytic cleavage by PARL is reduced."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 79; /note="L->D: Proteolytic cleavage by PARL is abolished."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 80; /note="A->D: Proteolytic cleavage by PARL is abolished."; /evidence="ECO:0000269|PubMed:28821867"; MUTAGEN 86..94; /note="DEERIQEEE->AAARIQAAA: Not cleaved by PARL. Not released in the cytosol."; /evidence="ECO:0000269|PubMed:29301859"; MUTAGEN 86..88; /note="DEE->AAA: No effect on cleavage by PARL. Not released in the cytosol."; /evidence="ECO:0000269|PubMed:29301859"; MUTAGEN 92..94; /note="EEE->AAA: No effect on cleavage by PARL. Not released in the cytosol."; /evidence="ECO:0000269|PubMed:29301859"; MUTAGEN 189; /note="R->Q: Abolishes binding to phosphocholine and ubiquinone."; /evidence="ECO:0000269|PubMed:36658222
uniprot
proteins
post_translational_modification
PTM: Proteolytically cleaved by YME1L1 and PARL (PubMed:20042613, PubMed:28288130, PubMed:28821867, PubMed:29301859). Mitochondrial targeting sequence is cleaved off by the mitochondrial processing peptidase YME1L1 in the mitochondrial matrix, generating an intermediate form. The intermediate form is cleaved within the transmembrane segment by the intramembrane protease PARL, producing the mature form of STARD7 which is released from the inner mitochondrial membrane and undergoes retro-translocation to the cytosol and/or internalization into the mitochondrion intermembrane space (IMS) (PubMed:28821867, PubMed:29301859). It seems that cleavage by PARL doesn't take place in myoblasts (By similarity). {ECO:0000250|UniProtKB:Q8R1R3, ECO:0000269|PubMed:20042613, ECO:0000269|PubMed:28288130, ECO:0000269|PubMed:28821867, ECO:0000269|PubMed:29301859}.
uniprot
proteins
pubmed
15013637
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15815621
uniprot
proteins
pubmed
20042613
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
25980009
uniprot
proteins
pubmed
28288130
uniprot
proteins
pubmed
28401922
uniprot
proteins
pubmed
28821867
uniprot
proteins
pubmed
29301859
uniprot
proteins
pubmed
32071354
uniprot
proteins
pubmed
34416390
uniprot
proteins
pubmed
36658222
uniprot
proteins
sequence_length
370
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: [StAR-related lipid transfer protein 7, mitochondrial]: Mitochondrion outer membrane {ECO:0000269|PubMed:20042613, ECO:0000269|PubMed:28821867}; Single-pass membrane protein {ECO:0000269|PubMed:28821867}; Cytoplasmic side {ECO:0000269|PubMed:28821867}.; SUBCELLULAR LOCATION: [StAR-related lipid transfer protein 7, mature]: Mitochondrion intermembrane space {ECO:0000269|PubMed:28821867, ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:36658222}. Cytoplasm, cytosol {ECO:0000269|PubMed:20042613, ECO:0000269|PubMed:28821867, ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:36658222}. Note=Precursor form is imported into mitochondria via the TOM and TIM23 complexes. After sequential cleavages by YME1L1 and PARL, mature form is released from the inner membrane and dynamically localizes to the cytosol and mitochondrial intermembrane space, where it associates transiently with the outer mitochondrial membrane to mediate phosphatidylcholine (PC) transfer between mitochondrial membranes. {ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:36658222}.
uniprot
proteins
transmembrane_region
TRANSMEM 60..96; /note="Helical"; /evidence="ECO:0000269|PubMed:28821867
uniprot
proteins