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OSBPL2

Function

Intracellular transport protein that binds sterols and phospholipids and mediates lipid transport between intracellular compartments. Increases plasma membrane cholesterol levels and decreases phosphatidylinositol-4,5-bisphosphate levels in the cell membrane (PubMed:30581148). Binds phosphoinositides, such as phosphatidylinositol-4,5-bisphosphate (PubMed:30581148). Exhibits strong binding to phosphatidic acid and weak binding to phosphatidylinositol 3-phosphate (PubMed:11279184). Binds cholesterol, dehydroergosterol, 22(R)-hydroxycholesterol and 25-hydroxycholesterol (in vitro) (PubMed:17428193, PubMed:19224871, PubMed:30581148).

Involvement in disease

Deafness, autosomal dominant, 67

DFNA67

A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the OSBP family.

Tissue Specificity

Widely expressed.

Cellular localization

Alternative names

KIAA0772, ORP2, OSBPL2, Oxysterol-binding protein-related protein 2, ORP-2, OSBP-related protein 2

swissprot:Q9H1P3 entrezGene:9885 omim:606731