Glycoprotein Ib
Glycoprotein Ib | ||
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according to PDB 1GWB | ||
other names |
Antigen CD42b, CD42b, platelet glycoprotein Ib |
|
Properties of human protein | ||
Mass / length primary structure | 652 amino acids + 206 amino acids, 21,718 Da + 71,540 Da | |
Secondary to quaternary structure | Heterodimer | |
Identifier | ||
External IDs | ||
Orthologue (human) | ||
Entrez | 953 | |
Ensemble | ENSG00000138185 | |
UniProt | P07359 | |
Refseq (mRNA) | NM_001098175.1 | |
Refseq (protein) | NP_001091645.1 | |
PubMed search |
953
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Glycoprotein Ib (synonymous CD42 , GP-Ib) is a surface protein on platelets .
properties
GP Ib is involved in blood clotting . It binds to the A1 domain of the Von Willebrand factor . It binds factor IX laterally and intracellularly to ABP-280 . It is glycosylated , phosphorylated, and sulfated . A mutation in GP Ib is responsible for Bernard-Soulier syndrome . A gain-of-function mutation creates the platelet type of Von Willebrand disease .
Web links
Individual evidence
- ↑ D. Shang, Z. Zhang, Q. Wang, Y. Ran, TS Shaw, JN Van, Y. Peng: Membrane skeleton orchestrates the platelet glycoprotein (GP) Ib-IX complex clustering and signaling. In: IUBMB life. Volume 68, Number 10, October 2016, pp. 823-829, doi : 10.1002 / iub.1559 , PMID 27634617 .
- ↑ LM Xu, GB Sun, P. Wang, N. Sun, QP Hu, FF Cao, B. Peng, DH Zhang: Single novel mutation in transmembrane region of glycoprotein (GP) IX affects GP Ib- IX complex expression and causes Bernard -Soulier syndrome. In: British Journal of Hematology . Volume 150, Number 5, September 2010, pp. 627-629, doi : 10.1111 / j.1365-2141.2010.08241.x , PMID 20497174 .
- ^ McPherson & Pincus: Henry's Clinical Diagnosis and Management by Laboratory Methods , 21st Edition, pp. 760-2 (WB Saunders, 2006).