Product Details

Purity

Greater than 85% as determined by SDS-PAGE.

Target Names

GP1BB

Uniprot No.

P13224

Research Area

Others

Alternative Names

GP1BB; Platelet glycoprotein Ib beta chain; GP-Ib beta; GPIb-beta; GPIbB; Antigen CD42b-beta; CD antigen CD42c

Species

Homo sapiens (Human)

Source

in vitro E.coli expression system

Expression Region

26-206aa

Target Protein Sequence

CPAPCSCAGTLVDCGRRGLTWASLPTAFPVDTTELVLTGNNLTALPPGLLDALPALRTAHLGANPWRCDCRLVPLRAWLAGRPERAPYRDLRCVAPPALRGRLLPYLAEDELRAACAPGPLCWGALAAQLALLGLGLLHALLLVLLLCRLRRLRARARARAAARLSLTDPLVAERAGTDES
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.

Mol. Weight

39.3 kDa

Protein Length

Full Length of Mature Protein

Tag Info

N-terminal 10xHis-SUMO-tagged and C-terminal Myc-tagged

Form

Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Buffer

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

3-7 business days

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet & COA

Please contact us to get it.

Target Background

Function

Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.

Gene References into Functions

Rare variants in GP1BB are responsible for autosomal dominant macrothrombocytopenia. PMID: 28064200
Data show that localization of the GP Ib-IX complex to the lipid domain is mediated by GP Ibbeta and GP IX transmembrane domains. PMID: 26203189
Low levels of CD9 coincidental with a novel nonsense mutation in glycoprotein Ibbeta in a patient with Bernard-Soulier syndrome. PMID: 26275786
a suspicion of 22q11.2 deletion is warranted in pediatric BSS patients with a mutation in the GPIbbeta gene, even without remarkable symptoms. PMID: 23566026
GPIbbeta missense mutations from Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Mutations A108P and P74R were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX surface expression PMID: 21908432
A novel variant of Bernard Soulier syndrome is described in which Ser23 of GPIbbeta is substituted by a Stop codon causing a premature termination of translation. PMID: 19484238
A 14-month-old boy with Bernard-Soulier syndrome was found to be homozygous for a nonsense mutation (c.423C > A) in the glycoprotein Ib-beta. PMID: 22343686
Identify TRAF4 as a novel binding partner for GPIb-IX-V and GPVI in human platelets. PMID: 20946164
putative convex surface of the LRR domain in GPIX is sufficient, in the context of full-length subunit, to mediate its association with GPIbbeta PMID: 19566547
Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3 PMID: 11812775
Role of GPIbbeta in modulating vWF mediated platelet adhesion. PMID: 11816713
A GP1BB missense mutation suppresses GPIb/IX complex expression and is found to cause Bernard-Soulier Syndrome in the homozygous form and giant platelets in the heterozygous form. PMID: 11816714
The cysteine knot of platelet GPIb beta is critical for the interaction of GPIb beta with GPIX. PMID: 12036872
PKA-mediated phosphorylation of GPIbbeta at Ser(166) negatively regulates VWF binding to GPIb-IX and is one of the mechanisms by which PKA mediates platelet inhibition PMID: 12361948
The GPIb beta intracellular domain has a role in controlling the adhesive properties of the GPIb/V/IX complex through phosphorylation of GPIb beta Ser166 and point to the existence of cross-talk between the GPIb beta and GPIb alpha intracellular domains. PMID: 12522011
novel hemizygous variant of Bernard-Soulier Syndrome in which Pro29 in one GPIbbeta allele is substituted by a Leu PMID: 12529755
To determine the sequence in the beta3 cytoplasmic domain that is critical to integrin signaling, cell lines were established that coexpress the platelet receptor for GP1BB. PMID: 12860973
identified novel GPIbbeta mutation is responsible for the Bernard-Soulier syndrome phenotype and provide an explanation for the molecular mechanism underlying the reduced platelet content of GPIb-IX complex in the heterozygous individuals studied PMID: 12958615
The SEPT5 gene resides approximately 250 nucleotides 5' to the GP Ibbeta gene and has been associated with modulating exocytosis from neurons and platelets as part of a presynaptic protein complex PMID: 15213102
Velocardiofacial syndrome patients have in-vitro defects in platelet function that may increase their risk of bleeding during surgery. PMID: 18064328
the N1421K substitution in the VWF affects the GPIb binding site or a recognition element by a conformational change of the A1 domain. PMID: 18637125
proplatelet formation in human megakaryocytes undergoes a complex spatio-temporal regulation orchestrated by adhesive proteins, GPIb-IX-V and myosin IIA PMID: 18752571
novel Ser 23 Stop mutation in GPIbbeta is responsible of BSS in the studied family and hampers the complex to form on the platelets surface. PMID: 18825380

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Involvement in disease

Bernard-Soulier syndrome (BSS)

Subcellular Location

Membrane; Single-pass type I membrane protein.

Tissue Specificity

Expressed in heart and brain.

Database Links

HGNC: 4440

OMIM: 138720

KEGG: hsa:2812

STRING: 9606.ENSP00000383382

UniGene: Hs.283743

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Code	CSB-CF009686HU
MSDS	MSDS
Size	$1620
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Image	(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Recombinant Human Platelet glycoprotein Ib beta chain (GP1BB)

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