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Rabbit Anti-PROSAAS/FITC Conjugated antibody
background:
PCSK1N is a 260 amino acid protein that is both secreted and localized to the trans-Golgi network. Expressed in pancreas and brain, PCSK1N is thought to play a role in the control of the neuroendocrine secretory pathway and may also be involved in PCSK1 inhibition. The gene encoding PCSK1N maps to human chromosome X, which contains nearly 153 million base pairs and houses over 1,000 genes. In conjunction with chromosome Y, chromosome X is responsible for sex determination, as an X and a Y chromosome lead to normal male development, while two copies of an X chromosome lead to normal female development. There are a number of conditions related to an abnormal number and combination of sex chromosomes, some of which include Turner's syndrome, color blindness, hemophilia and Duchenne muscular dystrophy.
Function:
May function in the control of the neuroendocrine secretory pathway. Proposed be a specific endogenous inhibitor of PCSK1. ProSAAS and Big PEN-LEN, both containing the SLCterminal inhibitory domain, but not the further processed peptides reduce PCSK1 activity in the endoplasmic reticulum and Golgi. It reduces the activity of the 84 kDa form but not the autocatalytically derived 66 kDa form of PCSK1. Subsequent processing of proSAAS may eliminate the inhibition. Slows down convertase-mediated processing of proopiomelanocortin and proenkephalin. May control the intracellular timing of PCSK1 rather than its total level of activity. The function of the processed secreted peptides is not known.
Subunit:
Interacts via the SLCterminal inhibitory domain with PCSK1 66 kDa form
Subcellular Location:
Secreted. Golgi apparatus > trans-Golgi network. A N-terminal processed peptide, probably Big SAAS or Little SAAS, is accumulated in cytoplasmic protein tau deposits in frontotemporal dementia and parkinsonism linked to chromosome 17 (Pick disease), Alzheimer disease and amyotrophic lateral sclerosis-parkinsonism/dementia complex 1.
Tissue Specificity:
Expressed in brain and pancreas.
Post-translational modifications:
Proteolytically cleaved in the Golgi.
O-glycosylated with a core 1 or possibly core 8 glycan.
Database links:
Entrez Gene: 27344 Human
Omim: 300399 Human
SwissProt: Q9UHG2 Human
Unigene: 522128 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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