细胞色素b5还原酶3抗体

CYB5R3 Antibody

Cat#：PL0306851

Isotype:Rabbit Polyclonal IgG

Immunogen：Peptide derived from human CYB5R3.

Purification: Immunogen affinity purified

Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)

Background：

CYB5R3 is a 301 amino acid protein encoded by the human gene CYB5R3. CYB5R3 belongs to the flavoprotein pyridine nucleotide cytochrome reductase family and has two naturally occuring isoforms. Isoform 1 is anchored to the cytoplasmic side of the endoplasmic reticulum membrane and mitochondrion outer membrane, while isoform 2 is the soluble form found in erythrocytes. CYB5R3 is involved in the desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism and, in erythrocytes, methemoglobin reduction. A serine residue at position 117 seems to only be found in persons of African origin. The allele frequency is 0.23 in African Americans. It is not found in Caucasians, Asians, Indo-Aryans or Arabs. This difference seems to have no effect on the enzyme activity. Defects in CYB5R3 are the cause of hereditary methemoglobinemia (HM). There are three forms of this disease: type 1 (HM1), in which the enzyme is only deficient in erythrocytes with a mild cyanosis; type 2 (HM2), in which the enzyme is completely deficient; and type 3 (HM3), where the deficiency is seen in all blood cells.

Specificity Reaction：Hu, Mo, Rat, Chi, Dog, Pig, Cow, Hor, Sheep

Application Notes:WB: 1:100-500 E: 1:500-1000 IHC: 1:100-500 IF: 1:100-500 ICC: 1:100-500

Storage ：

Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.

This product for research use only, not for use in humans, therapeutic or diagnostic procedures.

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