Thrombomodulin Active Protein | THBD active protein

Thrombomodulin, Recombinant, Human (CD141, TM, Fetomodulin)

Cat. Num. AAA637707

• Gene Names: THBD; TM; THRM; AHUS6; BDCA3; CD141; THPH12
• Purity: Highly Purified; 95%, as determined by SDS-PAGE and visualized by silver stain.
• Synonyms: Thrombomodulin; Recombinant; Human (CD141; TM; Fetomodulin); THBD active protein

• Purity/Purification: Highly Purified; 95%, as determined by SDS-PAGE and visualized by silver stain.
• Form/Format: Supplied as a liquid in 25mM Tris, 0.15M sodium chloride, pH 7.5.

• Activity: Measured by its ability to mediate thrombin activation of protein C. See Activity Assay Protocol for details. The specific activity of rhProtein C, measured with 5 ng of rhThrombin, 5 ng of rhTHBD, 100 ng of rhProtein C, and 0.1 mM Boc-D(OBzl)PR-AMC in 100 L of volume at room temperature, is > 300 pmoles/min/ g.
• Endotoxin Level: < 1.0 EU per 1 g of the protein as determined by the LAL method.
• Preparation and Storage: This protein can be aliquoted and stored under sterile conditions at -20 degree C in a manual defrost freezer for up to six months without a significant loss of activity.

Related Product Information for THBD active protein

Encoded by the THBD gene, thrombomodulin is also known as CD141 antigen. The deduced amino acid sequence of human THBD (Accession # NP_000352) predicts a signal peptide (aa 1-18) and a mature chain (aa 19-575) that consists of following domains: C-type lectin (aa 35-169), EGF-like (aa 242-281, aa 284-324, aa 325-363, aa 365-405, aa 404-440, and aa 441-481), Ser/Thr-rich (aa 490-506), transmembrane (aa 516 to 539) and cytoplasmic (aa 540-575) (1). rhTHBD consists of aa 19-515, corresponding to the extracellular portion of the type I membrane protein. Predominantly synthesized by vascular endothelial cells, THBD inhibits coagulation and fibrinolysis (1-3). It functions as a cell surface receptor and an essential cofactor for active thrombin, which in turn activates protein C and thrombin-activatable fibrinolysis inhibitor (TAFI), also known as carboxypeptidase B2 (CPB2). Activated protein C (APC), facilitated by protein S, degrades coagulation factors Va and VIIIa, which are required for thrombin activation. Activated CPB2 cleaves basic C-terminal amino acid residues of its substrates, including fibrin, preventing the conversion of plasminogen to plasmin. In addition, THBD gene polymorphisims are associated with human disease and THBD plays a role in thrombosis, stroke, arteriosclerosis, and cancer (4). For example, increased serum levels of THBD, due to protease cleavage, have been associated with smoking, cardiac surgery, atherosclerosis, liver cirrhosis, diabetes mellitus, cerebral and myocardial infarction, and multiple sclerosis (5).

Product Categories/Family for THBD active protein

Molecular Biology; MB-Coagulation Factors

NCBI and Uniprot Product Information

• NCBI GI #: 4507483
• NCBI GeneID: 7056
• NCBI Accession #: NP_000352.1
• NCBI GenBank Nucleotide #: NM_000361.2
• UniProt Accession #: P07204; Q8IV29; Q9UC32
• Molecular Weight: The purified, secreted rhTHBD has a N-terminal sequence of A19PAEPQPGGS. The 503 amino acid residue rhTHBD predicts a molecular mass of 53 kD and migrates with an apparent molecular mass of 95 kD in SDS-PAGE under reducing conditions.
• NCBI Official Full Name: thrombomodulin
• NCBI Official Synonym Full Names: thrombomodulin
• NCBI Official Symbol: THBD
• NCBI Official Synonym Symbols: TM; THRM; AHUS6; BDCA3; CD141; THPH12
• NCBI Protein Information: thrombomodulin; fetomodulin; CD141 antigen
• UniProt Protein Name: Thrombomodulin
• Protein Family: Thrombomodulin
• UniProt Gene Name: THBD
• UniProt Synonym Gene Names: THRM; TM
• UniProt Entry Name: TRBM_HUMAN

NCBI Description

The protein encoded by this intronless gene is an endothelial-specific type I membrane receptor that binds thrombin. This binding results in the activation of protein C, which degrades clotting factors Va and VIIIa and reduces the amount of thrombin generated. Mutations in this gene are a cause of thromboembolic disease, also known as inherited thrombophilia. [provided by RefSeq, Jul 2008]

Uniprot Description

thrombomodulin: Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated. Defects in THBD are the cause of thrombophilia due to thrombomodulin defect (THPH12). A hemostatic disorder characterized by a tendency to thrombosis. Defects in THBD are a cause of susceptibility to hemolytic uremic syndrome atypical type 6 (AHUS6). An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: 20p11.2

Cellular Component: extracellular space; cell surface; integral to plasma membrane; plasma membrane

Molecular Function: protein binding; transmembrane receptor activity; receptor activity; calcium ion binding

Biological Process: response to cAMP; negative regulation of fibrinolysis; negative regulation of blood coagulation; response to lipopolysaccharide; female pregnancy; blood coagulation; signal transduction; leukocyte migration; response to X-ray

Disease: Thrombophilia Due To Thrombomodulin Defect; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 6