MPI, belongs to the mannose-6-phosphate isomerase type 1 family, and is expressed in all tissues, more abundantly in heart, brain and skeletal muscle. Localized to the cytoplasm, MPI utilizes zinc as a cofactor and catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate. Mutations in the MPI gene were found in patients with carbohydrate-deficient glycoprotein syndrome, type Ib.
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