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Anti-HCCS Antibody Picoband® Fluoro488 Conjugated

Product Specifications

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.

Synonyms

Insulin-like growth factor-binding protein 1; IBP-1; IGF-binding protein 1; IGFBP-1; Igfbp1; Igfbp-1

Gene Name

HCCS

Gene ID

3052

UniProt

P53701

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

High expression in adult thyroid, lower expression in adult and fetal kidney and fetal brain. Not expressed in other tissues.

Applications

Flow Cytometry

Field of Research

Atherosclerosis, Cancer, Cardiovascular, Diabetes-associated, Growth Factors, Growth Factors/Hormones, Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

IGF-binding proteins prolong the half-life of the IGFs and have been shown to either inhibit or stimulate the growth promoting effects of the IGFs on cell culture. They alter the interaction of IGFs with their cell surface receptors. Promotes cell migration (By similarity) . .

References & Citations

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.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

27904 MW

Applications Notes

6

Gene Name Synonym

Insulin-like growth factor binding protein 1

Subcellular Location

Secreted.

Protein Name

Insulin-like growth factor-binding protein 1

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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