NCBI Description
This gene encodes a vitamin K-dependent plasma glycoprotein. The encoded protein is cleaved to its activated form by the thrombin-thrombomodulin complex. This activated form contains a serine protease domain and functions in degradation of the activated forms of coagulation factors V and VIII. Mutations in this gene have been associated with thrombophilia due to protein C deficiency, neonatal purpura fulminans, and recurrent venous thrombosis.[provided by RefSeq, Dec 2009]
Uniprot Description
PROC: Protein C is a vitamin K-dependent serine protease that regulates blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids. Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal dominant (THPH3). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Individuals with decreased amounts of protein C are classically referred to as having type I protein C deficiency and those with normal amounts of a functionally defective protein as having type II deficiency. Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal recessive (THPH4). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. It results in a thrombotic condition that can manifest as a severe neonatal disorder or as a milder disorder with late-onset thrombophilia. The severe form leads to neonatal death through massive neonatal venous thrombosis. Often associated with ecchymotic skin lesions which can turn necrotic called purpura fulminans, this disorder is very rare. Belongs to the peptidase S1 family.
Protein type: EC 3.4.21.69; Apoptosis; Protease
Chromosomal Location of Human Ortholog: 2q13-q14
Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; extracellular region; Golgi apparatus; Golgi lumen
Molecular Function: protein binding; serine-type endopeptidase activity
Biological Process: blood coagulation; ER to Golgi vesicle-mediated transport; leukocyte migration; negative regulation of apoptosis; negative regulation of blood coagulation; negative regulation of coagulation; negative regulation of inflammatory response; peptidyl-glutamic acid carboxylation; signal peptide processing
Disease: Thrombophilia Due To Protein C Deficiency, Autosomal Dominant; Thrombophilia Due To Protein C Deficiency, Autosomal Recessive
Product Notes
The PROC proc (Catalog #
AAA1267610) is a cDNA Clone and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: atgtggcagc tcacaagcct cctgctgttc gtggccacct ggggaatttc cggcacacca gctcctcttg actcagtgtt ctccagcagc gagcgtgccc accaggtgct gcggatccgc aaacgtgcca actccttcct ggaggagctc cgtcacagca gcctggagcg ggagtgcata gaggagatct gtgacttcga ggaggccaag gaaattttcc aaaatgtgga tgacacactg gccttctggt ccaagcacgt cgacggtgac cagtgcttgg tcttgccctt ggagcacccg tgcgccagcc tgtgctgcgg gcacggcacg tgcatcgacg gcatcggcag cttcagctgc gactgccgca gcggctggga gggccgcttc tgccagcgcg aggtgagctt cctcaattgc tctctggaca acggcggctg cacgcattac tgcctagagg aggtgggctg gcggcgctgt agctgtgcgc ctggctacaa gctgggggac gacctcctgc agtgtcaccc cgcagtgaag ttcccttgtg ggaggccctg gaagcggatg gagaagaagc gcagtcacct gaaacgagac acagaagacc aagaagacca agtagatccg cggctcattg atgggaagat gaccaggcgg ggagacagcc cctggcaggt ggtcctgctg gactcaaaga agaagctggc ctgcggggca gtgctcatcc acccctcctg ggtgctgaca gcggcccact gcatggatga gtccaagaag ctccttgtca ggcttggaga gtatgacctg cggcgctggg agaagtggga gctggacctg gacatcaagg aggtcttcgt ccaccccaac tacagcaaga gcaccaccga caatgacatc gcactgctgc acctggccca gcccgccacc ctctcgcaga ccatagtgcc catctgcctc ccggacagcg gccttgcaga gcgcgagctc aatcaggccg gccaggagac cctcgtgacg ggctggggct accacagcag ccgagagaag gaggccaaga gaaaccgcac cttcgtcctc aacttcatca agattcccgt ggtcccgcac aatgagtgca gcgaggtcat gagcaacatg gtgtctgaga acatgctgtg tgcgggcatc ctcggggacc ggcaggatgc ctgcgagggc gacagtgggg ggcccatggt cgcctccttc cacggcacct ggttcctggt gggcctggtg agctggggtg agggctgtgg gctccttcac aactacggcg tttacaccaa agtcagccgc tacctcgact ggatccatgg gcacatcaga gacaaggaag ccccccagaa gagctgggca ccttag.
It is sometimes possible for the material contained within the vial of
"PROC, cDNA Clone" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
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