Anti-HCCS Antibody Picoband®

SKU: A00924-2

Size: 100 μg

Storage: Store 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 freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,ICC,IF,Flow Cytometry,ELISA

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

Gene Name: HCCS

Specificity:

Background: Cytochrome c-type heme lyase is an enzyme that in humans is encoded by the HCCS gene on chromosome X. The protein encoded by this gene is an enzyme that covalently links a heme group to the apoprotein of cytochrome c. Defects in this gene are a cause of microphthalmia syndromic type 7 (MCOPS7). Three transcript variants encoding the same protein have been found for this gene.

Immunogen: E.coli-derived human HCCS recombinant protein (Position: K76-S268).

Clonality: Polyclonal

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

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Indrieri, A., Conte, I., Chesi, G., Romano, A., Quartararo, J., Tate, R., Ghezzi, D., Zeviani, M., Goffrini, P., Ferrero, I., Bovolenta, P., Franco, B. The impairment of HCCS leads to MLS syndrome by activating a non-canonical cell death pathway in the brain and eyes. EMBO Molec. Med. 5: 280-293, 2013. Note: Erratum: EMBO Molec. Med. 6: 849 only, 2014. 2. Morleo, M., Pramparo, T., Perone, L., Gregato, G., Le Caignec, C., Mueller, R. F., Ogata, T., Raas-Rothschild, A., de Blois, M. C., Wilson, L. C., Zaidman, G., Zuffardi, O., Ballabio, A., Franco, B. Microphthalmia with linear skin defects (MLS) syndrome: clinical, cytogenetic, and molecular characterization of 11 cases. Am. J. Med. Genet. 137A: 190-198, 2005. 3. Prakash, S. K., Cormier, T. A., McCall, A. E., Garcia, J. J., Sierra, R., Haupt, B., Zoghbi, H. Y., Van den Veyver, I. B. Loss of holocytochrome c-type synthetase causes the male lethality of X-linked dominant microphthalmia with linear skin defects (MLS) syndrome. Hum. Molec. Genet. 11: 3237-3248, 2002.

Uniprot ID: P53701

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: 31 kDa

Calculated Molecular Weight: 27904 MW

Gene ID: 3052

Protein Name: Holocytochrome c-type synthase

Gene Full Name: holocytochrome c synthase

Synonyms: CCHL; Cytochrome c type heme lyase; HCCS; Holocytochrome c type synthase; MCOPS7