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anti-BBS1 antibody :: Rabbit anti-Human, Mouse BBS1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS7050799
Unit / Price
0.05 mg  /  $160 +1 FREE 8GB USB
0.1 mg  /  $235 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

BBS1, Polyclonal Antibody

★Popular Item★
 Also Known As   

BBS1 Antibody

 Product Synonym Names    Bardet-Biedl syndrome 1 protein; BBS2-like protein 2; BBS1; BBS2L2
 Product Gene Name   

anti-BBS1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    AF503941 mRNA
 3D Structure    ModBase 3D Structure for Q8NFJ9
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse
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 Purity/Purification    >95%, Protein G purified
 Form/Format    Liquid
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 Immunogen    Recombinant Human Bardet-Biedl syndrome 1 protein (217-417AA)
 Preservative    0.03% Proclin 300
 Constituents    50% Glycerol, 0.01M PBS, PH 7.4
 Conjugation    Non-conjugated
 Santa Cruz Alternative    Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-49789 / sc-49790 / sc-49791
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-BBS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for anti-BBS1 antibody

   The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization.
 Applications Tested/Suitable for anti-BBS1 antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

 Application Notes for anti-BBS1 antibody    WB: 1: 500-1: 5000
IHC: 1: 20-1: 200
IF: 1: 50-1: 200
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 Western Blot (WB) of anti-BBS1 antibody    Western Blot
Positive WB detected in:Mouse lung tissue
All lanes: BBS1 antibody at 1ug/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 66,70,50 kDa
Observed band size: 66 kDa

anti-BBS1 antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-BBS1 antibody    Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7050799 at dilution of 1:100
anti-BBS1 antibody Immunohistochemistry (IHC) (IHC) image
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 Immunohistochemistry (IHC) of anti-BBS1 antibody    Immunohistochemistry of paraffin-embedded human liver tissue using MBS7050799 at dilution of 1:100
anti-BBS1 antibody Immunohistochemistry (IHC) (IHC) image
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 Immunofluorescence (IF) of anti-BBS1 antibody    Immunofluorescent analysis of HepG2 cells using MBS7050799 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
anti-BBS1 antibody Immunofluorescence (IF) image
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NCBI/Uniprot data below describe general gene information for BBS1. It may not necessarily be applicable to this product.
 NCBI GI #    28395045
 NCBI GeneID    582
 NCBI Accession #    NP_078925.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_024649.4 [Other Products]
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 UniProt Primary Accession #    Q8NFJ9 [Other Products]
 UniProt Secondary Accession #    Q32MM9; Q32MN0; Q96SN4 [Other Products]
 UniProt Related Accession #    Q8NFJ9 [Other Products]
 Molecular Weight    49,006 Da
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 NCBI Official Full Name    Bardet-Biedl syndrome 1 protein
 NCBI Official Synonym Full Names    Bardet-Biedl syndrome 1
 NCBI Official Symbol    BBS1 [Similar Products]
 NCBI Official Synonym Symbols   
BBS2L2
[Similar Products]
 NCBI Protein Information    Bardet-Biedl syndrome 1 protein
 UniProt Protein Name    Bardet-Biedl syndrome 1 protein
 UniProt Synonym Protein Names   
BBS2-like protein 2
 Protein Family    Bardet-Biedl syndrome 1 protein
 UniProt Gene Name    BBS1 [Similar Products]
 UniProt Synonym Gene Names    BBS2L2 [Similar Products]
 UniProt Entry Name    BBS1_HUMAN
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 NCBI Summary for BBS1    Mutations in this gene have been observed in patients with the major form (type 1) of Bardet-Biedl syndrome. The encoded protein may play a role in eye, limb, cardiac and reproductive system development. [provided by RefSeq, Jul 2008]
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 UniProt Comments for BBS1    BBS1: defects in BBS1 are a cause of Bardet-Biedl syndrome type (BBS). BBS is characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Two alternatively splioced isoforms have been reported.

Protein type: Unknown function

Chromosomal Location of Human Ortholog: 11q13

Cellular Component: axoneme; centrosome; cytosol

Molecular Function: patched binding; protein binding; smoothened binding

Biological Process: Golgi to plasma membrane protein transport; photoreceptor cell maintenance; retinal homeostasis

Disease: Bardet-biedl Syndrome 1
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 Research Articles on BBS1    1. M390R mutation in BBS1 reduces surface expression of insulin receptor in fibroblasts derived from BBS patients.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-BBS1 antibodyPathways associated with anti-BBS1 antibody
 Reference Product  PubMed Publications
 BBS2 antibody  >53 publications with BBS1 and BBS2
 BBS4 antibody  >52 publications with BBS1 and BBS4
 BBS7 antibody  >23 publications with BBS1 and BBS7
 BBS5 antibody  >20 publications with BBS1 and BBS5
 ARL6 antibody  >9 publications with BBS1 and ARL6
 BBS9 antibody  >8 publications with BBS1 and BBS9
 TTC8 antibody  >5 publications with BBS1 and TTC8
 BBIP1 antibody  >1 publications with BBS1 and BBIP1
 Products by Pathway  Pathway Diagram
 BBSome-mediated Cargo-targeting To Cilium Pathway antibodies  BBSome-mediated Cargo-targeting To Cilium Pathway Diagram
 Cargo Trafficking To The Periciliary Membrane Pathway antibodies  Cargo Trafficking To The Periciliary Membrane Pathway Diagram
 Cilium Assembly Pathway antibodies  Cilium Assembly Pathway Diagram
 Organelle Biogenesis And Maintenance Pathway antibodies  Organelle Biogenesis And Maintenance Pathway Diagram
Diseases associated with anti-BBS1 antibodyOrgans/Tissues associated with anti-BBS1 antibody
 Disease Name  Pubmed Publications
 Bardet-Biedl Syndrome Antibodies  >129 publications with BBS1 and Bardet-Biedl Syndrome
 Hypertension Antibodies  >13 publications with BBS1 and Hypertension
 Kidney Diseases Antibodies  >10 publications with BBS1 and Kidney Diseases
 Cardiovascular Diseases Antibodies  >7 publications with BBS1 and Cardiovascular Diseases
 Death Antibodies  >5 publications with BBS1 and Death
 Fibrosis Antibodies  >3 publications with BBS1 and Fibrosis
 Infertility, Male Antibodies  >3 publications with BBS1 and Infertility, Male
 Weight Loss Antibodies  >3 publications with BBS1 and Weight Loss
 Atrophy Antibodies  >2 publications with BBS1 and Atrophy
 Learning Disorders Antibodies  >2 publications with BBS1 and Learning Disorders
 Organ/Tissue Name  Pubmed Publications
 Eye Antibodies  >30 publications with BBS1 and Eye
 Kidney Antibodies  >24 publications with BBS1 and Kidney
 Heart Antibodies  >14 publications with BBS1 and Heart
 Brain Antibodies  >9 publications with BBS1 and Brain
 Blood Antibodies  >7 publications with BBS1 and Blood
 Liver Antibodies  >5 publications with BBS1 and Liver
 Stomach Antibodies  >4 publications with BBS1 and Stomach
 Muscle Antibodies  >2 publications with BBS1 and Muscle
 Embryonic Tissue Antibodies  >2 publications with BBS1 and Embryonic Tissue
 Adipose Tissue Antibodies  >2 publications with BBS1 and Adipose Tissue
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