Cardiovascular Antibodies

Anti-GP1BA Antibody (CAB16048)

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SKU:
CAB16048
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cardiovascular

Description

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Antibody Name:Anti-GP1BA Antibody
Antibody SKU:CAB16048
Antibody Size:20uL, 50uL, 100uL
Application:IF
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 19-259 of human GP1BA (NP_000164.5).
Application:IF
Recommended Dilution:IF 1:50 - 1:100
Reactivity:Human
Positive Samples:
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 19-259 of human GP1BA (NP_000164.5).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:ICEV SKVA SHLE VNCD KRNL TALP PDLP KDTT ILHL SENL LYTF SLAT LMPY TRLT QLNL DRCE LTKL QVDG TLPV LGTL DLSH NQLQ SLPL LGQT LPAL TVLD VSFN RLTS LPLG ALRG LGEL QELY LKGN ELKT LPPG LLTP TPKL EKLS LANN NLTE LPAG LLNG LENL DTLL LQEN SLYT IPKG FFGS HLLP FAFL HGNP WLCN CEIL YFRR WLQD NAEN VYVW KQGV DVKA MTSN V
Gene ID:2811
Uniprot:P07359
Cellular Location:Membrane, Single-pass type I membrane protein
Calculated MW:71kDa
Observed MW:Refer to figures
Synonyms:GP1BA, BDPLT1, BDPLT3, BSS, CD42B, CD42b-alpha, DBPLT3, GP1B, GPIbA, GPIbalpha, VWDP
Background:Glycoprotein Ib (GP Ib) is a platelet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that is linked by disulfide bonds. The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial platelet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the platelet that lead to enhanced platelet activation, thrombosis, and hemostasis. This gene encodes the alpha subunit. Mutations in this gene result in Bernard-Soulier syndromes and platelet-type von Willebrand disease. The coding region of this gene is known to contain a polymophic variable number tandem repeat (VNTR) domain that is associated with susceptibility to nonarteritic anterior ischemic optic neuropathy.
UniProt Protein Function:GPIbA: GP-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to the A1 domain of vWF, which is already bound to the subendothelium. Genetic variations in GP1BA may be a cause of susceptibility to non-arteritic anterior ischemic optic neuropathy (NAION). NAION is an ocular disease due to ischemic injury to the optic nerve. It usually affects the optic disk and leads to visual loss and optic disk swelling of a pallid nature. Visual loss is usually sudden, or over a few days at most and is usually permanent, with some recovery possibly occurring within the first weeks or months. Patients with small disks having smaller or non-existent cups have an anatomical predisposition for non-arteritic anterior ischemic optic neuropathy. As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage. Defects in GP1BA are a cause of Bernard-Soulier syndrome (BSS); also known as giant platelet disease (GPD). BSS patients have unusually large platelets and have a clinical bleeding tendency. Defects in GP1BA are the cause of benign mediterranean macrothrombocytopenia (BMM); also known as autosomal dominant benign Bernard-Soulier syndrome. BMM is characterized by mild or no clinical symptoms, normal platelet function, and normal megakaryocyte count. Defects in GP1BA are the cause of pseudo-von Willebrand disease (VWDP). A bleeding disorder is caused by an increased affinity of GP-Ib for soluble vWF resulting in impaired hemostatic function due to the removal of vWF from the circulation.
UniProt Protein Details:

Protein type:Membrane protein, integral; Cell surface; Cell adhesion

Chromosomal Location of Human Ortholog: 17p13.2

Cellular Component: anchored to external side of plasma membrane; cell surface; membrane; integral to plasma membrane; plasma membrane

Molecular Function:protein binding; thrombin receptor activity

Biological Process: platelet activation; fibrinolysis; cell surface receptor linked signal transduction; regulation of blood coagulation; cell morphogenesis; cell adhesion; blood coagulation; blood coagulation, intrinsic pathway

Disease: Pseudo-von Willebrand Disease; Bernard-soulier Syndrome; Bernard-soulier Syndrome, Type A2, Autosomal Dominant; Nonarteritic Anterior Ischemic Optic Neuropathy, Susceptibility To

NCBI Summary:Glycoprotein Ib (GP Ib) is a platelet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that is linked by disulfide bonds. The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial platelet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the platelet that lead to enhanced platelet activation, thrombosis, and hemostasis. This gene encodes the alpha subunit. Mutations in this gene result in Bernard-Soulier syndromes and platelet-type von Willebrand disease. The coding region of this gene is known to contain a polymophic variable number tandem repeat (VNTR) domain that is associated with susceptibility to nonarteritic anterior ischemic optic neuropathy. [provided by RefSeq, Oct 2013]
UniProt Code:P07359
NCBI GenInfo Identifier:121531
NCBI Gene ID:2811
NCBI Accession:P07359.1
UniProt Secondary Accession:P07359,Q14441, Q16469, Q8N1F3, Q8NG39, Q9HDC7, Q9UEK1 Q9UQS4,
UniProt Related Accession:P07359
Molecular Weight:17.4kDa
NCBI Full Name:Platelet glycoprotein Ib alpha chain
NCBI Synonym Full Names:glycoprotein Ib (platelet), alpha polypeptide
NCBI Official Symbol:GP1BA  
NCBI Official Synonym Symbols:BSS; GP1B; VWDP; CD42B; GPIbA; BDPLT1; BDPLT3; DBPLT3; CD42b-alpha  
NCBI Protein Information:platelet glycoprotein Ib alpha chain; GP-Ib alpha; antigen CD42b-alpha; platelet membrane glycoprotein 1b-alpha subunit
UniProt Protein Name:Platelet glycoprotein Ib alpha chain
UniProt Synonym Protein Names:Antigen CD42b-alpha
Protein Family:Envelope glycoprotein
UniProt Gene Name:GP1BA  
UniProt Entry Name:GP1BA_HUMAN
Anti-GP1BA Antibody (CAB16048)Immunofluorescence analysis of THP-1 cells using GP1BA Polyclonal Antibody (CAB16048) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
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