Anti-BBS8/TTC8 Antibody Picoband®, HRP Conjugate

• 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:

  pyrophosphatase (inorganic) 1

• 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

• 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. .

• Components:

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

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

• Calculated Molecular Weight:

  51200 MW

• Observed Molecular Weight:

  23 kDa

• Fragment:

  Rabbit IgG

• Applications Notes:

  Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat
  Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
  ELISA, 0.1-0.5 μg/ml, -
  

• Subcellular Location:

  Cytoplasm.

• Protein Name:

  BAG family molecular chaperone regulator 5