MPDU1 Antibodies, cDNA Clones Research Reagents

MPDU1 (Mannose-P-Dolichol Utilization Defect 1, also known as CDGIF; PQLC5; PP3958; SLC66A5; HBEBP2BPA), located on 17p13.1, is a Protein Coding gene. This gene encodes an endoplasmic reticulum membrane protein that is required for utilization of the mannose donor mannose-P-dolichol in the synthesis of lipid-linked oligosaccharides and glycosylphosphatidylinositols. MPDU1 plays a role in the utilization of dolichol-phosphate-mannose (DPM). It belongs to the MPDU1 (TC 2.A.43.3) family. MPDU1 mutations underlie a novel human congenital disorder of glycosylation, designated type If.

MPDU1 Antibody (1)

    MPDU1 cDNA Clone (41)

    XM_006721598.1

    クローニングベクター cDNA 製品

    In lentiviral vector

    NM_011900.4

    クローニングベクター cDNA 製品

    In lentiviral vector

    NM_001266148.1

    クローニングベクター cDNA 製品

    In lentiviral vector

    MPDU1 の背景知識

    Mannose-phosphate-dolichol utilization defect 1 (MPDU1) plays a role in the utilization of dolichol-phosphate-mannose (DPM). CDG-If is caused by a defect in the gene MPDU1, the human homolog of hamster Lec35, and is the first disorder to affect the use, rather than the biosynthesis, of donor substrates for lipid-linked oligosaccharides. This leads to the synthesis of incomplete and poorly transferred precursor oligosaccharides lacking both mannose and glucose residues. Kato III cells lacking MPDU1 have all the hallmarks of a glycosylation-deficient cell line, including altered sensitivity to lectins and the formation of truncated Lipid-linked Oligosaccharides (LLOs). Kato III cells and the MPDU1-rescued Kato IIIM cells provide a novel model to examine the consequences of defective LLO biosynthesis both in vitro and in vivo.

    MPDU1 の参考文献

    • van Tol W, et al. (2019) A mutation in mannose-phosphate-dolichol utilization defect 1 reveals clinical symptoms of congenital disorders of glycosylation type i and dystroglycanopathy. JIMD Rep 50 (1): 31-39.
    • Kranz C, et al. (2001) A mutation in the human mpdu1 gene causes congenital disorder of glycosylation type if (cdg-if). J Clin Invest 108 (11): 1613-1619.
    • Bennett DC, et al. (2018) Mpdu1 regulates ceacam1 and cell adhesion in vitro and in vivo. Glycoconj J 35 (3): 265-274.

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