Anti-HLCS Antibody Picoband®

SKU: A02377-1

Size: 100 μg

Storage: 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.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Rat
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml

Gene Name: HLCS

Specificity:

Background: This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.

Immunogen: E.coli-derived human HLCS recombinant protein (Position: Q14-K654). Human HLCS shares 75.9% amino acid (aa) sequence identity with mouse HLCS.

Clonality: Polyclonal

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

Purification: Immunogen affinity purified.

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

Reference: 1. Aoki, Y., Li, X., Sakamoto, O., Hiratsuka, M., Akaishi, H., Xu, L., Briones, P., Suormala, T., Baumgartner, E. R., Suzuki, Y., Narisawa, K. Identification and characterization of mutations in patients with holocarboxylase synthetase deficiency. Hum. Genet. 104: 143-148, 1999. 2. Aoki, Y., Suzuki, Y., Li, X., Sakamoto, O., Chikaoka, H., Takita, S., Narisawa, K. Characterization of mutant holocarboxylase synthetase (HCS): a Km for biotin was not elevated in a patient with HCS deficiency. Pediat. Res. 42: 849-854, 1997. 3. Dupuis, L., Leon-Del-Rio, A., Leclerc, D., Campeau, E., Sweetman, L., Saudubray, J. M., Herman, G., Gibson, K. M., Gravel, R. A. Clustering of mutations in the biotin-binding region of holocarboxylase synthetase in biotin-responsive multiple carboxylase deficiency. Hum. Molec. Genet. 5: 1011-1016, 1996.

Uniprot ID: P50747

Host: Rabbit

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

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 81 kDa

Calculated Molecular Weight: 81 kDa

Gene ID: 3141

Protein Name: Biotin--protein ligase

Gene Full Name: holocarboxylase synthetase

Synonyms: Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--[methylmalonyl-CoA-carboxytransferase] ligase; Biotin--[methylcrotonoyl-CoA-carboxylase] ligase; Biotin--[acetyl-CoA-carboxylase] ligase; Biotin apo-protein ligase