RGD Reference Report - Membrane-bound cytochrome b5 reductase (methemoglobin reductase) in human erythrocytes. Study in normal and methemoglobinemic subjects. - Rat Genome Database

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Membrane-bound cytochrome b5 reductase (methemoglobin reductase) in human erythrocytes. Study in normal and methemoglobinemic subjects.

Authors: Choury, D  Leroux, A  Kaplan, JC 
Citation: Choury D, etal., J Clin Invest. 1981 Jan;67(1):149-55.
RGD ID: 11352693
Pubmed: PMID:7451647   (View Abstract at PubMed)
PMCID: PMC371582   (View Article at PubMed Central)
DOI: DOI:10.1172/JCI110007   (Journal Full-text)

In this study we present evidence that in human erythrocytes NADH-cytochrome b5 reductase (methemoglobin reductase) is not only soluble but also tightly bound to the membrane. The membrane methemoglobin reductase-like activity is unmasked by Triton X-100 treatment, and represents about half of the total activity in the erythrocytes. Like the amphiphilic microsomal-bound cytochrome b5 reductase, the erythrocyte membrane-bound enzyme is solubilized by cathepsin D. Because this treatment is effective on unsealed ghosts but not on resealed (inside-in) ghosts, it is concluded that the enzyme is strongly bound to the inner face of the membrane. The erythrocyte membrane enzyme is antigenically similar to the soluble enzyme. The two forms of enzyme are specified by the same gene, in that both were found defective in six patients with recessive congenital methemoglobinemia. We suggest that the cytochrome b5 reductase of the erythrocyte membrane is the primary gene product. A posttranslational partial proteolysis probably gives rise to the soluble form of the enzyme, which serves as a methemoglobin reductase.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Congenital Methemoglobinemia  IEP 11352693protein:decreased activity:erythrocyte membrane:RGD 
Congenital Methemoglobinemia  ISOCYB5A (Homo sapiens)11352693; 11352693protein:decreased activity:erythrocyte membrane:RGD 

Objects Annotated

Genes (Rattus norvegicus)
Cyb5a  (cytochrome b5 type A)

Genes (Mus musculus)
Cyb5a  (cytochrome b5 type A (microsomal))

Genes (Homo sapiens)
CYB5A  (cytochrome b5 type A)


Additional Information