[HTML][HTML] Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality
C DeRossi, L Bode, EA Eklund, F Zhang… - Journal of Biological …, 2006 - ASBMB
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 mannose to
rescue the anticipated defective glycosylation. Surprisingly, although glycosylation was …
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 mannose to
rescue the anticipated defective glycosylation. Surprisingly, although glycosylation was …