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Article

Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality.

The Journal of biological chemistry - 3 Mar 2006

DeRossi Charles, Bode Lars, Eklund Erik A, Zhang Fangrong, Davis Joseph A, Westphal Vibeke, Wang Ling, Borowsky Alexander D, Freeze Hudson H

Abstract excerpt

MPI encodes phosphomannose isomerase, which interconverts fructose 6-phosphate and mannose 6-phosphate (Man-6-P), used for glycoconjugate biosynthesis. MPI mutations in humans impair protein glycosylation causing congenital disorder of glycosylation Ib (CDG-Ib), but oral mannose supplements normalize glycosylation. To establish a mannose-responsive mouse model for CDG-Ib, we ablated Mpi and provided dams with...

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Identifiers

PMID: 16339137
DOI: 10.1074/jbc.M511982200

Genes

MPI

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

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Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality.PMID 16339137Read ↗

A zebrafish model of congenital disorders of glycosylation with phosphomannose isomerase deficiency reveals an early opportunity for corrective mannose supplementation.Semantic proximity Read ↗

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Targeted disruption of the mouse phosphomannomutase 2 gene causes early embryonic lethality.Semantic proximity Read ↗

Agm1/Pgm3-mediated sugar nucleotide synthesis is essential for hematopoiesis and development.Semantic proximity Read ↗

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