Anti-BBS8/TTC8 Antibody Picoband®

CAT: 0519-A07486-2Size: 100 µg/VialDry Ice: NoHazardous: No

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CAT#:0519-A07486-2Size:100 µg/Vial

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24/48H Stock Items & 2 to 6 Weeks non Stock Items.

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Background

Tetratricopeptide repeat domain 8 (TTC8) also known as Bardet–Biedl syndrome 8 is a protein that in humans is encoded by the TTC8 gene.This gene encodes a protein that has been ly linked to Bardet-Biedl syndrome. The primary features of this syndrome include retinal dystrophy, obesity, polydactyly, renal abnormalities and learning disabilities. Experimentation in non-human eukaryotes suggests that this gene is expressed in ciliated cells and that it is involved in the formation of cilia. A mutation in this gene has also been implicated in nonsyndromic retinitis pigmentosa. Alternative splicing results in multiple transcript variants.

Description

Boster Bio Anti-BBS8/TTC8 Antibody Picoband® catalog # A07486-2. Tested in ELISA, Flow Cytometry, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Synonyms

Inorganic pyrophosphatase; Pyrophosphate phospho-hydrolase; Ppase; PPA1; IOPPP; PP

Gene Name

TTC8

Gene ID

123016

UniProt

Q8TAM2

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human BBS8/TTC8 recombinant protein (Position: E271-Q533) .

Clonality

Polyclonal

Tissue Specificity

Expressed ubiquitously.

Applications

WB, Flow Cytometry, ELISA

Field of Research

Cancer, Cofactors, Vitamins/Minerals, Metabolism, Pathways and Processes, Signal Transduction, Vitamins/Minerals

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Function

Inhibits both auto-ubiquitination of PARK2 and ubiquitination of target proteins by PARK2 (By similarity) . May function as a nucleotide exchange factor for HSP/HSP70, promoting ADP release, and activating Hsp70-mediated refolding. .

References & Citations

1. Ansley, S. J., Badano, J. L., Blacque, O. E., Hill, J., Hoskins, B. E., Leitch, C. C., Kim, J. C., Ross, A. J., Eichers, E. R., Teslovich, T. M., Mah, A. K., Johnsen, R. C., Cavender, J. C., Lewis, R. A., Leroux, M. R., Beales, P. L., Katsanis, N. Basal body dysfunction is a likely cause of pleiotropic Bardet-Biedl syndrome. Nature 425: 628-633, 2003. 2. Goyal, S., Jager, M., Robinson, P. N., Vanita, V. Confirmation of TTC8 as a disease gene for nonsyndromic autosomal recessive retinitis pigmentosa (RP51) . Clin. Genet. 89: 454-460, 2016. 3. Jin, H., White, S. R., Shida, T., Schulz, S., Aguiar, M., Gygi, S. P., Bazan, J. F., Nachury, M. V. The conserved Bardet-Biedl syndrome proteins assemble a coat that traffics membrane proteins to cilia. Cell 141: 1208-1219, 2010.

Storage Conditions

At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Calculated Molecular Weight

51200 MW

Observed Molecular Weight

23 kDa

Applications Notes

Gene Name Synonym

Pyrophosphatase (inorganic) 1

Subcellular Location

Cytoplasm.

Protein Name

BAG family molecular chaperone regulator 5

Isotype

Rabbit IgG

Contents

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

UniProtKB · Q8TAM2

Tetratricopeptide repeat protein 8

TTC8_HUMAN · Homo sapiens

View on UniProt ↗

Primary accession: Q8TAM2
Review status: UniProtKB reviewed (Swiss-Prot)
Gene: TTC8
Protein existence: 1: Evidence at protein level
Organism: Homo sapiens (Human)
Taxonomy ID: 9606
Alternative names: TPR repeat protein 8
EC number: —
Processing: —
Secondary accessions: A6NFG2, B3KWA5, Q67B97, Q86SY0, Q86TV9, Q86U26, Q8NDH9, Q96DG8

Protein keywords

Coding sequence diversity

Alternative splicing

Disease

Bardet-Biedl syndromeCiliopathyDisease variantIntellectual disabilityObesityRetinitis pigmentosa

Cellular component

Cell membraneCell projectionCiliumCytoplasmCytoskeletonMembrane

Biological process

Cilium biogenesis/degradationProtein transportTransport

Technical term

Proteomics identificationReference proteome

Domain

RepeatTPR repeat

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