Product Name :
Cox-10 (K147) Peptide Applications :
Blocking Background :
Defects in COX10 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; 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. Alternative Name :
Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase; COX10 Swiss-Prot :
Q12887 Product :
1 mg/ml in DI water. Purification&Purity :
Synthetic peptide Cox-10 (K147). (Note: the amino acid sequence is proprietary). The purity is > 98%. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
This peptide can be used with studies using BS2868 Cox-10 (K147) pAb.
Cox-10 (K147) Peptide Applications :
Blocking Background :
Defects in COX10 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; 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. Alternative Name :
Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase; COX10 Swiss-Prot :
Q12887 Product :
1 mg/ml in DI water. Purification&Purity :
Synthetic peptide Cox-10 (K147). (Note: the amino acid sequence is proprietary). The purity is > 98%. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
This peptide can be used with studies using BS2868 Cox-10 (K147) pAb.
Blocking peptide available as BS2868PP