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NBEAL2

Function

Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis.

Involvement in disease

Gray platelet syndrome

GPS

A rare platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with mild to moderate bleeding tendency and moderate thrombocytopenia. The platelets are enlarged and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules.

None

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

Sequence Similarities

Belongs to the WD repeat neurobeachin family.

Tissue Specificity

Expressed in megakaryocytes.

Cellular localization

Alternative names

KIAA0540, UNQ253/PRO290, NBEAL2, Neurobeachin-like protein 2

swissprot:Q6ZNJ1 omim:614169 entrezGene:23218