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AKAP9

Domain

RII-binding site, predicted to form an amphipathic helix, could participate in protein-protein interactions with a complementary surface on the R-subunit dimer.

Function

Scaffolding protein that assembles several protein kinases and phosphatases on the centrosome and Golgi apparatus. Required to maintain the integrity of the Golgi apparatus (PubMed:10202149, PubMed:15047863). Required for microtubule nucleation at the cis-side of the Golgi apparatus (PubMed:15047863, PubMed:19242490). Required for association of the centrosomes with the poles of the bipolar mitotic spindle during metaphase (PubMed:25657325). In complex with PDE4DIP isoform 13/MMG8/SMYLE, recruits CAMSAP2 to the Golgi apparatus and tethers non-centrosomal minus-end microtubules to the Golgi, an important step for polarized cell movement (PubMed:27666745, PubMed:28814570). In complex with PDE4DIP isoform 13/MMG8/SMYLE, EB1/MAPRE1 and CDK5RAP2, contributes to microtubules nucleation and extension also from the centrosome to the cell periphery (PubMed:29162697).

Isoform 4

Associated with the N-methyl-D-aspartate receptor and is specifically found in the neuromuscular junction (NMJ) as well as in neuronal synapses, suggesting a role in the organization of postsynaptic specializations.

Involvement in disease

Long QT syndrome 11

LQT11

A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy.

None

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

Tissue Specificity

Widely expressed (PubMed:10202149). Isoform 4: Highly expressed in skeletal muscle and in pancreas (PubMed:9482789).

Cellular localization

Alternative names

AKAP350, AKAP450, KIAA0803, AKAP9, A-kinase anchor protein 9, AKAP-9, A-kinase anchor protein 350 kDa, A-kinase anchor protein 450 kDa, AKAP 120-like protein, Centrosome- and Golgi-localized PKN-associated protein, Protein hyperion, Protein kinase A-anchoring protein 9, Protein yotiao, AKAP 350, hgAKAP 350, AKAP 450, CG-NAP, PRKA9

swissprot:Q99996 omim:604001 entrezGene:10142