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HYOU1

Function

Has a pivotal role in cytoprotective cellular mechanisms triggered by oxygen deprivation. May play a role as a molecular chaperone and participate in protein folding.

Involvement in disease

Immunodeficiency 59 and hypoglycemia

IMD59

An autosomal recessive primary immunologic disorder characterized by combined immunodeficiency, granulocytopenia, B-cell and dendritic cell deficiency, recurrent septic infections of the respiratory tract, skin and mucous membranes, and disturbed glucose metabolism.

None

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

Sequence Similarities

Belongs to the heat shock protein 70 family.

Tissue Specificity

Highly expressed in tissues that contain well-developed endoplasmic reticulum and synthesize large amounts of secretory proteins. Highly expressed in liver and pancreas and lower expression in brain and kidney. Also expressed in macrophages within aortic atherosclerotic plaques, and in breast cancers.

Cellular localization

Alternative names

GRP170, HSPH4, ORP150, HYOU1, Hypoxia up-regulated protein 1, 150 kDa oxygen-regulated protein, 170 kDa glucose-regulated protein, Heat shock protein family H member 4, ORP-150, GRP-170

swissprot:Q9Y4L1 omim:601746 entrezGene:10525