Signal Transduction Antibodies 2

Anti-NBEAL2 Antibody (CAB15797)

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SKU:
CAB15797
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-NBEAL2 Antibody
Antibody SKU:CAB15797
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1800-2100 of human NBEAL2 (NP_055990.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:HeLa
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1800-2100 of human NBEAL2 (NP_055990.1).
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:LLHW GALW RQLA SPCG AWAL RDTP IPRW KLSS AETY SRMR LKLV PNHH FDPH LEAS ALRD NLGE VPLT PTEE ASLP LAVT KEAK VSTP PELL QEDQ LGED ELAE LETP MEAA ELDE QREK LVLS AECQ LVTV VAVV PGLL EVTT QNVY FYDG STER VETE EGIG YDFR RPLA QLRE VHLR RFNL RRSA LELF FIDQ ANYF LNFP CKVG TTPV SSPS QTPR PQPG PIPP HTQV RNQV YSWL LRLR PPSQ GYLS SRSP QEML RASG LTQK WVQR EISN FEYL MQLN TIAG RTYN DLSQ YPVF P
Gene ID:23218
Uniprot:Q6ZNJ1
Cellular Location:Endoplasmic reticulum
Calculated MW:282kDa/299kDa/302kDa
Observed MW:302kDa
Synonyms:NBEAL2, BDPLT4, GPS
Background:The protein encoded by this gene contains a beige and Chediak-Higashi (BEACH) domain and multiple WD40 domains, and may play a role in megakaryocyte alpha-granule biogenesis. Mutations in this gene are a cause of gray platelet syndrome.
UniProt Protein Function:NBEAL2: Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis. Defects in NBEAL2 are the cause of gray platelet syndrome (GPS). GPS is a rare platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with mild to moderate bleeding tendency and moderate thrombocytopenia. The platelets are enlarged and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules. Belongs to the WD repeat neurobeachin family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Adaptor/scaffold

Chromosomal Location of Human Ortholog: 3p21.31

Cellular Component: endoplasmic reticulum; extrinsic to membrane

Molecular Function:phospholipid binding

Biological Process: platelet formation

Disease: Gray Platelet Syndrome

NCBI Summary:The protein encoded by this gene contains a beige and Chediak-Higashi (BEACH) domain and multiple WD40 domains, and may play a role in megakaryocyte alpha-granule biogenesis. Mutations in this gene are a cause of gray platelet syndrome. [provided by RefSeq, Dec 2011]
UniProt Code:Q6ZNJ1
NCBI GenInfo Identifier:149944548
NCBI Gene ID:23218
NCBI Accession:NP_055990.1
UniProt Secondary Accession:Q6ZNJ1,O60288, Q6P994, Q6UX91, Q8NAC9,
UniProt Related Accession:Q6ZNJ1
Molecular Weight:299,342 Da
NCBI Full Name:neurobeachin-like protein 2
NCBI Synonym Full Names:neurobeachin like 2
NCBI Official Symbol:NBEAL2  
NCBI Official Synonym Symbols:GPS; BDPLT4  
NCBI Protein Information:neurobeachin-like protein 2
UniProt Protein Name:Neurobeachin-like protein 2
Protein Family:Neurobeachin-like protein
UniProt Gene Name:NBEAL2  
UniProt Entry Name:NBEL2_HUMAN
Anti-NBEAL2 Antibody (CAB15797)Western blot analysis of extracts of HeLa cells, using NBEAL2 antibody (CAB15797) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
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