BS3019

antibody from Bioworld Technology, Inc
Targeting: HCCS CCHL, MLS
Provider product page for BS3019
Western blot
Immunocytochemistry
Immunohistochemistry

Antibody data

Product number
BS3019 - Provider product page
Provider
Bioworld Technology, Inc
Product name
CCHL (R104) polyclonal antibody
Antibody type
Polyclonal
Antigen
Synthetic peptide, corresponding to amino acids 80-120 of Human CCHL.
Description
CCHL (cytochrome c-type heme lyase), also known as HCCS (holocytochrome c-type synthase), is a 268 amino acid mitochondrial inner membrane protein that belongs to the cytochrome c-type heme lyase family. Containing two HRM (heme regulatory motif) repeats, HCCS participates in the covalent linkage of a heme group to an apoprotein of cytochrome c. The gene encoding HCCS maps to the human X chromosome. Defects to this gene cause microphthalmia syndromic type 7 (MCOPS7), also known as MIDAS syndrome or microphthalmia with linear skin defects (MLS). MCOPS7 is an X-linked male-lethal disorder that results in eye deformation, unilateral or bilateral microphthalmia, linear skin defects in affected females, and in utero lethality for males.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
HCCS protein structure - BS3019 shown in red.