GABARAPL2

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Description

The GABARAPL2 (GABA type A receptor associated protein like 2) is a protein-coding gene located on chromosome 16.

Gamma-aminobutyric acid receptor-associated protein-like 2 is a protein that in humans is encoded by the GABARAPL2 gene.

GABARAPL2, also known as GATE-16, is a ubiquitin-like modifier involved in intracellular protein trafficking, particularly within the Golgi apparatus. It modulates Golgi transport by coordinating the activity of NSF (N-ethylmaleimide-sensitive factor) and SNAREs (soluble NSF attachment protein receptors). This process involves stimulating NSF's ATPase activity, which in turn promotes its association with GOSR1. GABARAPL2 is also a key player in autophagy, a cellular process responsible for degrading and recycling damaged or unnecessary components. Specifically, GABARAPL2 is involved in mitophagy, the selective removal of mitochondria. It helps maintain mitochondrial health and energy production by ensuring proper mitochondrial turnover. Unlike LC3 proteins, which are involved in the initial stages of autophagosome formation, GABARAPL2 is essential for the later stages of autophagosome maturation.

GABARAPL2 is also known as ATG8, ATG8C, GATE-16, GATE16, GEF-2, GEF2.

Associated Diseases

• substance abuse
• hemoglobin D disease
• hemoglobin E-beta-thalassemia syndrome
• X-linked severe congenital neutropenia
• dominant beta-thalassemia
• alpha-thalassemia-myelodysplastic syndrome
• neutropenia, severe congenital, 2, autosomal dominant
• 22q11.2 deletion syndrome
• hereditary persistence of fetal hemoglobin-sickle cell disease syndrome
• hemoglobin H disease
• neutropenia, severe congenital, 1, autosomal dominant
• hemoglobin E disease
• hemoglobin C-beta-thalassemia syndrome
• X-linked sideroblastic anemia 1