Cell Biology Antibodies 14

Anti-BBS1 Antibody (CAB16773)

(No reviews yet) Write a Review
SKU:
CAB16773
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-BBS1 Antibody
Antibody SKU:CAB16773
Antibody Size:20uL, 50 uL
Application:IHC
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 240-370 of human BBS1 (NP_078925.3).
Application:IHC
Recommended Dilution:IHC 1:50 - 1:200
Reactivity:Human, Mouse
Positive Samples:
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 240-370 of human BBS1 (NP_078925.3).
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:AKMS LPSV PVFL EVSG QFDV EFRL AAAC RNGN IYIL RRDS KHPK YCIE LSAQ PVGL IRVH KVLV VGST QDSL HGFT HKGK KLWT VQMP AAIL TMNL LEQH SRGL QAVM AGLA NGEV RIYR DKAL LNVI HTP
Gene ID:582
Uniprot:Q8NFJ9
Cellular Location:
Calculated MW:49kDa/65kDa/69kDa
Observed MW:Refer to figures
Synonyms:BBS1, BBS2L2
Background: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.
UniProt Protein Function: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 (PubMed:17574030, PubMed:22072986). Plays a role in olfactory cilium biogenesis/maintenance and trafficking.
NCBI Summary: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]
UniProt Code:Q8NFJ9
NCBI GenInfo Identifier:28395045
NCBI Gene ID:582
NCBI Accession:NP_078925.3
UniProt Related Accession:Q8NFJ9
Molecular Weight:49kDa; 65kDa; 69kDa
NCBI Full Name:Bardet-Biedl syndrome 1 protein
NCBI Synonym Full Names:Bardet-Biedl syndrome 1
NCBI Official Symbol:BBS1  
NCBI Official Synonym Symbols:BBS2L2  
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  
View AllClose

0 Reviews

View AllClose

Related Products