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anti-COX10 antibody :: COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase Polyclonal Antibody

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Catalog # MBS716684
Unit / Price
  0.05 mL  /  $230 +1 FREE 8GB USB
  0.15 mL  /  $500 +1 FREE 8GB USB
anti-COX10 antibody
Product Name

COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase, Polyclonal Antibody

Also Known As

Rabbit anti-human COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast) polyclonal Antibody

Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
443
OMIM
602125
Clonality
Polyclonal
Isotype
IgG
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Immunogen
Human COX10
Storage Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-54061 / sc-54061 / sc-54062 / sc-134792
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of anti-COX10 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Applications Tested/Suitable for anti-COX10 antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for COX10. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
27,594 Da[Similar Products]
NCBI Official Full Name
COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast)
NCBI Official Synonym Full Names
cytochrome c oxidase assembly homolog 10 (yeast)
NCBI Official Symbol
COX10  [Similar Products]
NCBI Protein Information
protoheme IX farnesyltransferase, mitochondrial; heme O synthase; heme A: farnesyltransferase; cytochrome c oxidase subunit X; cytochrome c oxidase assembly protein; COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase
UniProt Protein Name
Protoheme IX farnesyltransferase, mitochondrial
UniProt Synonym Protein Names
Heme O synthase
UniProt Gene Name
COX10  [Similar Products]
UniProt Entry Name
COX10_HUMAN
NCBI Summary for COX10
Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion. [provided by RefSeq, Jul 2008]
UniProt Comments for COX10
COX10: Converts protoheme IX and farnesyl diphosphate to heme O. Defects in COX10 are a cause of mitochondrial complex IV deficiency (MT-C4D); also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Defects in COX10 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Belongs to the UbiA prenyltransferase family.

Protein type: Membrane protein, integral; EC 2.5.1.-; Transferase; Membrane protein, multi-pass; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 17p12

Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane

Molecular Function: cytochrome-c oxidase activity; farnesyltranstransferase activity; protoheme IX farnesyltransferase activity

Biological Process: mitochondrial fission; heme o biosynthetic process; respiratory chain complex IV assembly; cellular respiration; porphyrin metabolic process; aerobic respiration; mitochondrial electron transport, cytochrome c to oxygen; heme a biosynthetic process; heme biosynthetic process

Disease: Leigh Syndrome; Mitochondrial Complex Iv Deficiency
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

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