NAA10

N-alpha-acetylation is among the most common post-translational protein modifications in eukaryotic cells. This process involves the transfer of an acetyl group from acetyl-coenzyme A to the alpha-amino group on a nascent polypeptide and is essential for

Full Name

N(Alpha)-Acetyltransferase 10, NatA Catalytic Subunit

Function

Catalytic subunit of N-terminal acetyltransferase complexes which display alpha (N-terminal) acetyltransferase activity (PubMed:15496142, PubMed:19826488, PubMed:19420222, PubMed:20145209, PubMed:27708256, PubMed:25489052, PubMed:29754825, PubMed:20154145, PubMed:32042062).

Acetylates amino termini that are devoid of initiator methionine (PubMed:19420222).

The alpha (N-terminal) acetyltransferase activity may be important for vascular, hematopoietic and neuronal growth and development. Without NAA15, displays epsilon (internal) acetyltransferase activity towards HIF1A, thereby promoting its degradation (PubMed:12464182).

Represses MYLK kinase activity by acetylation, and thus represses tumor cell migration (PubMed:19826488).

Acetylates, and stabilizes TSC2, thereby repressing mTOR activity and suppressing cancer development (PubMed:20145209).

Acetylates HSPA1A and HSPA1B at 'Lys-77' which enhances its chaperone activity and leads to preferential binding to co-chaperone HOPX (PubMed:27708256).

Acetylates HIST1H4A (PubMed:29754825).

Acts as a negative regulator of sister chromatid cohesion during mitosis (PubMed:27422821).

Biological Process

DNA packaging Source: ProtInc
Internal protein amino acid acetylation Source: ProtInc
Negative regulation of maintenance of mitotic sister chromatid cohesion, centromeric Source: UniProtKB
N-terminal peptidyl-glutamic acid acetylation Source: GO_Central
N-terminal peptidyl-serine acetylation Source: GO_Central
N-terminal protein amino acid acetylation Source: UniProtKB
Protein acetylation Source: UniProtKB

Cellular Location

Cytoplasm
Nucleus
Note: Also present in the free cytosolic and cytoskeleton-bound polysomes.

Involvement in disease

N-terminal acetyltransferase deficiency (NATD):
An enzymatic deficiency resulting in postnatal growth failure with severe delays and dysmorphic features. It is clinically characterized by wrinkled forehead, prominent eyes, widely opened anterior and posterior fontanels, downsloping palpebral fissures, thickened lids, large ears, flared nares, hypoplastic alae, short columella, protruding upper lip, and microretrognathia. There are also delayed closing of fontanels and broad great toes. Skin is characterized by redundancy or laxity with minimal subcutaneous fat, cutaneous capillary malformations, and very fine hair and eyebrows. Death results from cardiogenic shock following arrhythmia.
Microphthalmia, syndromic, 1 (MCOPS1):
A rare syndrome defined by the canonical features of unilateral or bilateral microphthalmia or anophthalmia and defects in the skeletal and genitourinary systems. Microphthalmia is a disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues (anophthalmia). In many cases, microphthalmia/anophthalmia occurs in association with syndromes that include non-ocular abnormalities. Anomalies of the digits, teeth, and ears are hallmarks of MCOPS1. Intellectual disability ranges from mild to severe, with self-mutilating behaviors and seizures in severely affected MCOPS1 individuals.

PTM

Cleaved by caspases during apoptosis.
Phosphorylation by IKBKB/IKKB at Ser-209 promotes its proteasome-mediated degradation.
Autoacetylated at Lys-136 which stimulates its catalytic activity.