Bardet-Biedl syndrome 4; May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane [...]

ubiquitin C

CDP-diacylglycerol--inositol 3-phosphatidyltransferase; Catalyzes the biosynthesis of phosphatidylinositol (PtdIns) as well as PtdIns-inositol exchange reaction. May thus act to reduce an excessive cellular PtdIns content. The exchange activity is due to the reverse reaction of PtdIns synthase and is dependent on CMP, which is tightly bound to the enzyme

cardiolipin synthase 1; Catalyzes the reversible phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL) (diphosphatidylglycerol) and glycerol

CDP-diacylglycerol--inositol 3-phosphatidyltransferase; Catalyzes the biosynthesis of phosphatidylinositol (PtdIns) as well as PtdIns-inositol exchange reaction. May thus act to reduce an excessive cellular PtdIns content. The exchange activity is due to the reverse reaction of PtdIns synthase and is dependent on CMP, which is tightly bound to the enzyme

ubiquitin C

cardiolipin synthase 1; Catalyzes the reversible phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL) (diphosphatidylglycerol) and glycerol

CDP-diacylglycerol--inositol 3-phosphatidyltransferase; Catalyzes the biosynthesis of phosphatidylinositol (PtdIns) as well as PtdIns-inositol exchange reaction. May thus act to reduce an excessive cellular PtdIns content. The exchange activity is due to the reverse reaction of PtdIns synthase and is dependent on CMP, which is tightly bound to the enzyme

cardiolipin synthase 1; Catalyzes the reversible phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL) (diphosphatidylglycerol) and glycerol

tafazzin

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

sorting and assembly machinery component 50 homolog (S. cerevisiae); May be required for the assembly pathway of mitochondrial outer membrane proteins (By similarity)

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

translocase of outer mitochondrial membrane 20 homolog (yeast); Central component of the receptor complex responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with TOM22 functions as the transit peptide receptor at the surface of the mitochondrion outer membrane and facilitates the movement of preproteins into the TOM40 translocation pore (By similarity)

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

translocase of outer mitochondrial membrane 22 homolog (yeast); Central receptor component of the translocase of the outer membrane of mitochondria (TOM complex) responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with the peripheral receptor TOM20 functions as the transit peptide receptor and facilitates the movement of preproteins into the translocation pore

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

translocase of outer mitochondrial membrane 40 homolog (yeast); Channel-forming protein essential for import of protein precursors into mitochondria (By similarity)

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

translocase of outer mitochondrial membrane 7 homolog (yeast); Required for assembly and stability of the TOM complex

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae); Receptor that accelerates the import of all mitochondrial precursor proteins (By similarity)

CORO7-PAM16 readthrough; May play a role in the maintenance of the Golgi apparatus morphology and in the protein export from the Golgi

ubiquitin C

interferon-induced protein with tetratricopeptide repeats 1; Interferon-induced antiviral RNA-binding protein that specifically binds single-stranded RNA bearing a 5’-triphosphate group (PPP-RNA), thereby acting as a sensor of viral single- stranded RNAs and inhibiting expression of viral messenger RNAs. Single-stranded PPP-RNAs, which lack 2’-O-methylation of the 5’ cap and bear a 5’-triphosphate group instead, are specific from viruses, providing a molecular signature to distinguish between self and non-self mRNAs by the host during viral infection. Directly binds PPP-RNA in a non-se [...]

ubiquitin C

interferon-induced protein with tetratricopeptide repeats 5; Interferon-induced RNA-binding protein that specifically binds single-stranded RNA bearing a 5’-triphosphate group (PPP- RNA), thereby acting as a sensor of viral single-stranded RNAs. Single-stranded PPP-RNAs, which lack 2’-O-methylation of the 5’ cap and bear a 5’-triphosphate group instead, are specific from viruses, providing a molecular signature to distinguish between self and non-self mRNAs by the host during viral infection. Directly binds PPP-RNA in a non-sequence-specific manner. Also recognizes and binds tRNAs

ubiquitin C

metaxin 2; Involved in transport of proteins into the mitochondrion

metaxin 3; Could function in transport of proteins into the mitochondrion (By similarity)

metaxin 2; Involved in transport of proteins into the mitochondrion

sorting and assembly machinery component 50 homolog (S. cerevisiae); May be required for the assembly pathway of mitochondrial outer membrane proteins (By similarity)

metaxin 2; Involved in transport of proteins into the mitochondrion

translocase of outer mitochondrial membrane 20 homolog (yeast); Central component of the receptor complex responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with TOM22 functions as the transit peptide receptor at the surface of the mitochondrion outer membrane and facilitates the movement of preproteins into the TOM40 translocation pore (By similarity)

metaxin 2; Involved in transport of proteins into the mitochondrion

translocase of outer mitochondrial membrane 22 homolog (yeast); Central receptor component of the translocase of the outer membrane of mitochondria (TOM complex) responsible for the recognition and translocation of cytosolically synthesized mitochondrial preproteins. Together with the peripheral receptor TOM20 functions as the transit peptide receptor and facilitates the movement of preproteins into the translocation pore

metaxin 2; Involved in transport of proteins into the mitochondrion

translocase of outer mitochondrial membrane 40 homolog (yeast); Channel-forming protein essential for import of protein precursors into mitochondria (By similarity)

metaxin 2; Involved in transport of proteins into the mitochondrion

translocase of outer mitochondrial membrane 7 homolog (yeast); Required for assembly and stability of the TOM complex