TTC8 Antibody

Code CSB-PA025244GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
TTC8
Alternative Names
Bardet Biedl syndrome 8 protein antibody; Bardet Biedl syndrome type 8 antibody; Bardet-Biedl syndrome 8 protein antibody; BBS8 antibody; RP51 antibody; Tetratricopeptide repeat domain 8 antibody; Tetratricopeptide repeat protein 8 antibody; TPR repeat protein 8 antibody; TTC 8 antibody; Ttc8 antibody; TTC8_HUMAN antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human TTC8
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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.
Gene References into Functions
  1. A splice-site mutation in a retina-specific exon of TTC8 causes nonsyndromic retinitis pigmentosa. PMID: 20451172
  2. A homozygous null BBS8 mutation leads to Bardet-Biedl syndrome with randomization of left-right body axis symmetry, a known defect of the nodal cilium PMID: 14520415
Involvement in disease
Retinitis pigmentosa 51 (RP51); Bardet-Biedl syndrome 8 (BBS8)
Subcellular Location
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite. Cell projection, cilium.
Tissue Specificity
Widely expressed.
Database Links

HGNC: 20087

OMIM: 608132

KEGG: hsa:123016

STRING: 9606.ENSP00000370031

UniGene: Hs.303055

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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