Recombinant Human Collagen type IV alpha-3-binding protein (COL4A3BP)

Uniprot Id

Q9Y5P4

Target Species

Human

Target Name

CERT1

Target Full Name

Ceramide transfer protein

Target Function

Shelters ceramides and diacylglycerol lipids inside its START domain and mediates the intracellular trafficking of ceramides and diacylglycerol lipids in a non-vesicular manner.

Target Involvement

Mental retardation, autosomal dominant 34 (MRD34)

Target Subcellular Location

Cytoplasm. Golgi apparatus. Endoplasmic reticulum.

Target Tissue Specificity

Widely expressed.

Target Synonyms

Alpha 3 type IV collagen binding protein; C43BP_HUMAN; Ceramide transfer protein; Ceramide transporter; CERT; Cert protein; CERTL; col4a3bp; Collagen type IV alpha 3 (Goodpasture antigen) binding protein; Collagen type IV alpha 3 binding protein; Collagen type IV alpha-3-binding protein; FLJ20597; Goodpasture antigen binding protein; Goodpasture antigen-binding protein; GPBP; hCERT; Lipid transfer protein CERTL; Procollagen type IV alpha 3 (Goodpasture antigen) binding protein; StAR related lipid transfer (START) domain containing 11; StAR related lipid transfer protein 11; StAR-related lipid transfer protein 11; StARD 11; StARD11; START domain containing 11; START domain containing protein 11; START domain-containing protein 11

Target Background

This gene encodes a kinase that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, known as the Goodpasture antigen. Goodpasture disease is the result of an autoimmune response directed at this antigen. One isoform of this protein is also involved in ceramide intracellular transport. Three transcript variants encoding different isoforms have been found for this gene.