Name: Anti-MTCO1 Antibody (CAB17889)
Brand: Cell Biology Antibodies 7
SKU: CAB17889
Availability: OutOfStock

Antibody Name:	Anti-MTCO1 Antibody
Antibody SKU:	CAB17889
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant protein of human MTCO1.

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	HeLa, HepG2, 293T, THP-1

Immunogen:	Recombinant protein of human MTCO1.
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:	4512
Uniprot:	P00395
Cellular Location:
Calculated MW:	57kDa
Observed MW:	40kDa

Synonyms:	COI, MTCO1, COX1, MT-CO1
Background:

UniProt Protein Function:	COX1: Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B. Defects in MT-CO1 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. MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis. Defects in MT-CO1 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-CO1 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. Defects in MT-CO1 are a cause of deafness sensorineural mitochondrial (DFNM). DFNM is a form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies. Defects in MT-CO1 are a cause of colorectal cancer (CRC). Belongs to the heme-copper respiratory oxidase family.
UniProt Protein Details:	Protein type:Membrane protein, integral; Energy Metabolism - oxidative phosphorylation; Oxidoreductase; Membrane protein, multi-pass; EC 1.9.3.1; Mitochondrial Chromosomal Location of Human Ortholog: - Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Deafness, Nonsyndromic Sensorineural, Mitochondrial
UniProt Code:	P00395
NCBI GenInfo Identifier:	116977
NCBI Gene ID:	4512
NCBI Accession:	P00395.1
UniProt Related Accession:	P00395
Molecular Weight:
NCBI Full Name:	Cytochrome c oxidase subunit 1
NCBI Synonym Full Names:	mitochondrially encoded cytochrome c oxidase I
NCBI Official Symbol:	MT-CO1
NCBI Official Synonym Symbols:	COI; MTCO1; COX1
NCBI Protein Information:	cytochrome c oxidase subunit I
UniProt Protein Name:	Cytochrome c oxidase subunit 1
UniProt Synonym Protein Names:	Cytochrome c oxidase polypeptide I
Protein Family:	Cox1 intron-like protein
UniProt Gene Name:	MT-CO1
UniProt Entry Name:	COX1_HUMAN

	Western blot analysis of extracts of various cell lines, using MTCO1 antibody (CAB17889) 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 Enhanced Kit (RM00021). Exposure time: 15s.
	Immunohistochemistry of paraffin-embedded rat kidney using MTCO1 antibody (CAB17889) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human stomach using MTCO1 antibody (CAB17889) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse lung using MTCO1 antibody (CAB17889) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of NIH-3T3 cells using MTCO1 Polyclonal Antibody (CAB17889) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.