Anti-MT-CO3 Antibody (CAB17891)

Antibody Name:	Anti-MT-CO3 Antibody
Antibody SKU:	CAB17891
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Mouse
Host Species:	Rabbit
Immunogen:	Recombinant protein of human MT-CO3.

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:100 - 1:200
Reactivity:	Mouse
Positive Samples:	Mouse kidney

Immunogen:	Recombinant protein of human MT-CO3.
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	Email for sequence
Gene ID:	4514
Uniprot:	P00414
Cellular Location:
Calculated MW:	30kDa
Observed MW:	30KDa

Synonyms:	COIII, MTCO3, COX3, MT-CO3
Background:

UniProt Protein Function:	COX3: Subunits I, II and III form the functional core of the enzyme complex. Defects in MT-CO3 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Defects in MT-CO3 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 MT-CO3 are associated with recurrent myoglobinuria mitochondrial (RM-MT). Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Belongs to the cytochrome c oxidase subunit 3 family.
UniProt Protein Details:	Protein type:Oxidoreductase; Membrane protein, integral; Mitochondrial; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: - Disease: Leber Optic Atrophy; Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes
UniProt Code:	P00414
NCBI GenInfo Identifier:	6648058
NCBI Gene ID:	4514
NCBI Accession:	P00414.2
UniProt Secondary Accession:	P00414,Q14Y83,
Molecular Weight:	29,951 Da
NCBI Full Name:	Cytochrome c oxidase subunit 3
NCBI Synonym Full Names:	mitochondrially encoded cytochrome c oxidase III
NCBI Official Symbol:	MT-CO3
NCBI Official Synonym Symbols:	COIII; MTCO3; COX3
NCBI Protein Information:	cytochrome c oxidase III
UniProt Protein Name:	Cytochrome c oxidase subunit 3
UniProt Synonym Protein Names:	Cytochrome c oxidase polypeptide III
UniProt Gene Name:	MT-CO3
UniProt Entry Name:	COX3_HUMAN

Western blot analysis of extracts of Mouse kidney, using MT-CO3 Rabbit pAb (CAB17891) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 180s.

Antibodies	ELISA Kits
Anti-MT-CO3 Antibody (CAB18318)	Human MT-CO3 / COX3 ELISA Kit
	Human Cytochrome c oxidase subunit 3 (MT-CO3) ELISA Kit

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)