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protein

Adenylate kinase isoenzyme 1

aka AK 1

AK1
protein:P00568disease:adad:direction:up

Gene

AK1

Organism

Homo sapiens(9606)

Length

194 aa

Mass

21,635 Da

AI summarysource-grounded · cited inline
claude-haiku-4-5-20251001

Adenylate kinase isoenzyme 1 (AK1) is a 194-amino acid phosphotransferase that catalyzes reversible transfer of the terminal phosphate group between ATP and AMP, playing a central role in cellular energy homeostasis and adenine nucleotide metabolism (UniProt: P00568). The enzyme also displays broad nucleoside diphosphate kinase activity and can catalyze thiamine triphosphate synthesis at very low rates. AK1 is associated with congenital non-spherocytic hemolytic anemia (CNSHA3, MIM 612631), an autosomal recessive condition characterized by hemolytic anemia and erythrocyte adenylate kinase deficiency (UniProt: P00568).

AK1 is relevant to Alzheimer's Disease pathology. In human post-mortem AD brain tissue compared to age-matched controls, AK1 protein levels are elevated (mean log2FC = 1.0949) in subcellular fractions analyzed by TMT-labeled mass spectrometry (Chaparral AD proteomics). This upregulation may reflect compensatory responses to energy metabolism disruption or changes in subcellular compartmentalization characteristic of AD neurodegeneration.

Generated from the curated entity record below. May contain errors — verify against source links.

Proteomics Evidence · AD

↑ Up in AD

P3

+1.095

P2

not detected

S2

not detected

S3

not detected

Mean log₂FC across detected fractions: +1.0949 (1 of 4 fractions detected)

Human post-mortem AD brain vs age-matched controls, TMT-labeled, 4 subcellular fractions (P2, P3, S2, S3), DDA proteomics.

Related Publications

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Function

Catalyzes the reversible transfer of the terminal phosphate group between ATP and AMP. Also displays broad nucleoside diphosphate kinase activity. Plays an important role in cellular energy homeostasis and in adenine nucleotide metabolism (By similarity) (PubMed:21080915, PubMed:23416111, PubMed:2542324). Also catalyzes at a very low rate the synthesis of thiamine triphosphate (ThTP) from thiamine diphosphate (ThDP) and ADP (By similarity)

Disease associations

  • Anemia, congenital, non-spherocytic hemolytic, 3CNSHA3

    An autosomal recessive disease characterized by hemolytic anemia and undetectable erythrocyte adenylate kinase activity.

Sources

Last updated 5/8/2026, 6:35:31 AM