Affinage

GMDS

GDP-mannose 4,6 dehydratase · UniProt O60547

Length
372 aa
Mass
42.0 kDa
Annotated
2026-06-10
27 papers in source corpus 9 papers cited in narrative 9 extracted findings
Cross-family judge vs UniProt: tie faithfulness: 5/5 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

GMDS (GDP-mannose 4,6-dehydratase) catalyzes the first, rate-limiting and non-redundant step of the de novo GDP-fucose biosynthesis pathway, converting GDP-mannose toward GDP-fucose so that loss of GMDS abolishes intracellular GDP-fucose and cellular protein fucosylation (PMID:17559959, PMID:18047682). It collaborates functionally with the downstream fucosyltransferase FUT8 in the same fucosylation process, where co-depletion synergistically eliminates antibody fucosylation (PMID:18047682). Through its control of cellular fucosylation, GMDS gates extrinsic apoptotic death-receptor signaling: GMDS-deficient cells resist TRAIL/DR4-, DR5- and CD95-mediated apoptosis but not intrinsic drug-induced death, with the defect mapping specifically to assembly of the secondary FADD-dependent complex II (caspase-8/cFLIP) rather than primary DISC formation, and acting independently of direct death-receptor fucosylation (PMID:19361506, PMID:22027835). This apoptotic gating underlies a tumor-suppressive, immune-surveillance role: restoring GMDS sensitizes colon cancer cells to NK cell- and TRAIL-dependent killing and suppresses tumor growth and metastasis in vivo (PMID:19361506). Beyond cancer, GMDS is cell-intrinsically required for T-cell development past the double-positive thymic stage (PMID:40642090), and in model organisms it regulates Notch signaling — controlling Notch protein stability via the OFUT1 axis (PMID:21720678) and acting upstream of Notch in hair cell regeneration (PMID:41097001) — as well as retinal ganglion cell survival and ocular stress responses (PMID:40571142).

Mechanistic history

Synthesis pass · year-by-year structured walk · 9 steps
  1. 2007 High

    Established that GMDS is the rate-limiting, non-redundant entry enzyme of de novo GDP-fucose synthesis, defining it as the master switch for cellular fucosylation.

    Evidence siRNA knockdown and knockout in CHO cells with HPLC GDP-fucose quantification and antibody fucosylation analysis

    PMID:17559959 PMID:18047682

    Open questions at the time
    • Does not resolve the enzyme's structural/catalytic mechanism
    • Salvage-pathway contributions to GDP-fucose pools in other cell types not addressed
  2. 2007 Medium

    Placed GMDS and the fucosyltransferase FUT8 in the same fucosylation process by showing synergistic defucosylation upon co-depletion, distinguishing it from transporter-level control.

    Evidence siRNA double knockdown with flow cytometry fucosylation readout and ADCC assay

    PMID:18047682

    Open questions at the time
    • Epistasis inferred from siRNA combinations in a single cell line
    • Does not establish direct physical interaction between GMDS and FUT8
  3. 2009 High

    Connected GMDS-dependent fucosylation to extrinsic apoptosis and tumor immune surveillance, showing GMDS acts upstream of TRAIL signaling and NK cell-mediated killing.

    Evidence GMDS mutation/rescue in HCT116 colon cancer cells, TRAIL apoptosis assays, xenograft model, NK cytotoxicity with anti-TRAIL blocking antibody

    PMID:19361506

    Open questions at the time
    • Molecular target whose fucosylation enables TRAIL sensitivity not identified here
    • Generality beyond colon cancer not tested
  4. 2011 High

    Pinpointed the death-receptor signaling step GMDS controls, showing the block is at secondary complex II (caspase-8/cFLIP) assembly and independent of direct death-receptor fucosylation.

    Evidence Co-IP of DISC components, caspase-8 activity assays, western blot of complex II, DR4/DR5 fucosylation comparison

    PMID:22027835

    Open questions at the time
    • The specific fucosylated protein governing complex II assembly remains unidentified
    • Mechanism linking fucosylation status to FADD-dependent complex II formation not defined
  5. 2011 Medium

    Linked GMDS/GDP-fucose to Notch protein stability through OFUT1, extending GMDS function from apoptosis into developmental signaling.

    Evidence Drosophila genetics and epistasis with OFUT1 and Notch Abruptex mutants

    PMID:21720678

    Open questions at the time
    • Genetic epistasis in Drosophila; biochemical demonstration of fucose-dependent OFUT1 activity on Notch not shown
    • Conservation of the OFUT1-Notch mechanism in mammals not established
  6. 2018 Medium

    Demonstrated a cell-autonomous tumor-promoting requirement for GMDS in lung adenocarcinoma, nominating a CASP8-CDKN1A axis and reinforcing GMDS as upstream of apoptotic/cell-cycle control.

    Evidence shRNA knockdown in A549/H1299, proliferation/cell-cycle/apoptosis assays, xenograft, microarray transcriptome

    PMID:29843634

    Open questions at the time
    • CASP8-CDKN1A axis identified by transcriptomics, not mechanistically validated
    • Apparent pro-tumor role here contrasts with tumor-suppressive role in colon cancer; context dependence unexplained
  7. 2025 High

    Revealed a cell-intrinsic requirement for GMDS in thymic T-cell development past the double-positive stage, defining a non-redundant role in lymphopoiesis.

    Evidence ENU-mutant mouse, flow immunophenotyping, bone marrow reconstitution and mixed chimera experiments

    PMID:40642090

    Open questions at the time
    • Molecular fucosylation target driving the DP-stage block not identified
    • Whether the defect reflects altered Notch or TCR-associated signaling not resolved
  8. 2025 Medium

    Showed GMDS restrains hair cell number and regeneration upstream of Notch signaling, providing in vivo developmental evidence for the GMDS-Notch link.

    Evidence CRISPR/Cas9 gmds zebrafish, neomycin ablation, pharmacological Notch inhibition, live imaging

    PMID:41097001

    Open questions at the time
    • Notch placed downstream by pharmacology/epistasis, not by direct biochemistry
    • Single model system
  9. 2025 Medium

    Implicated GMDS dosage in retinal ganglion cell survival and ocular stress responses, broadening its physiological role to tissue maintenance.

    Evidence CRISPR/Cas9 gmds haploinsufficient zebrafish, RNA-seq, histological eye phenotyping

    PMID:40571142

    Open questions at the time
    • Stress-response and cell-death gene changes are correlative
    • Mechanistic link between reduced fucosylation and RGC loss not established

Open questions

Synthesis pass · forward-looking unresolved questions
  • The identity of the fucosylated protein(s) whose modification by the GMDS pathway controls complex II assembly, T-cell development, and Notch regulation remains the central open mechanistic question.
  • No direct fucosylated effector linking GMDS loss to complex II/caspase-8 signaling identified
  • Context-dependent tumor-suppressive vs tumor-promoting roles unreconciled
  • No structural/catalytic mechanism described in the corpus

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016491 oxidoreductase activity 1 GO:0016829 lyase activity 1
Pathway
R-HSA-392499 Metabolism of proteins 2 R-HSA-5357801 Programmed Cell Death 2 R-HSA-1430728 Metabolism 1
Partners

Evidence

Reading pass · 9 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2007 GMDS (GDP-mannose 4,6-dehydratase) encodes the first enzyme in the de novo GDP-fucose biosynthesis pathway; siRNA-mediated knockdown of GMDS in CHO cells reduced cellular fucosylation in a manner correlated with mRNA expression levels, and GMDS knockout cells were devoid of intracellular GDP-fucose and produced completely non-fucosylated antibodies, confirming GMDS is the rate-limiting enzyme in the de novo GDP-fucose synthesis pathway with no functional redundancy. siRNA knockdown, GMDS knockout cell line generation, GDP-fucose quantification by HPLC, antibody fucosylation analysis Journal of biotechnology High 17559959 18047682
2007 Simultaneous double knockdown of GMDS and FUT8 (alpha-1,6-fucosyltransferase) produced a synergistic effect on reduction of antibody fucosylation, achieving complete defucosylation, whereas knockdown of GMDS combined with GDP-fucose transporter (GFT) siRNA did not show the same synergistic effect, indicating that GMDS and FUT8 collaborate in the same intracellular fucosylation process. siRNA double knockdown, flow cytometry for fucosylation, ADCC assay BMC biotechnology Medium 18047682
2009 Loss-of-function mutations in GMDS in the human colon cancer cell line HCT116 result in virtually complete loss of cellular fucosylation; re-introduction of wild-type GMDS restored cellular fucosylation. GMDS-deficient cells were resistant to TRAIL-induced apoptosis, and GMDS-rescued cells transplanted into athymic mice showed dramatically suppressed tumor growth and metastasis through NK cell-mediated tumor surveillance. Anti-TRAIL blocking antibody suppressed the accelerated direct cell lysis of GMDS-rescued cells by splenocytes, placing GMDS upstream of TRAIL signaling in NK cell-mediated tumor immune surveillance. Mutational analysis, GMDS transfection/rescue, in vitro TRAIL apoptosis assay, xenograft mouse model, NK cell cytotoxicity assay, anti-TRAIL blocking antibody Gastroenterology High 19361506
2011 GMDS deficiency inhibits both DR4- and DR5-mediated (and CD95-mediated) apoptosis, but not intrinsic (drug-induced) apoptosis. Mechanistically, GMDS deficiency does not block primary DISC formation or caspase-8 recruitment/activation at the DISC, but specifically inhibits formation of the secondary FADD-dependent complex II (comprising caspase-8 and cFLIP) downstream of DISC. DR4 was found to be fucosylated while DR5 was not, yet GMDS deficiency blocked both pathways, indicating the block occurs independent of direct fucosylation of death receptors at the level of complex II assembly. Co-immunoprecipitation of DISC components, caspase-8 activity assay, western blot for complex II components, comparison of DR4/DR5 fucosylation status The Journal of biological chemistry High 22027835
2011 In Drosophila, GMD (the ortholog of human GMDS) activity regulates the stability of the Notch protein; low GMD expression leads to Notch degradation that is entirely dependent on OFUT1 (O-fucosyltransferase-1). The GDP-fucose/OFUT1 balance determines OFUT1's ability to endocytose and degrade Notch, placing GMD upstream of OFUT1-mediated Notch protein stability. Drosophila genetics (mutant analysis, UAS/Gal4 system), epistasis analysis with OFUT1 and Notch Abruptex mutants Biological research Medium 21720678
2018 GMDS knockdown in human lung adenocarcinoma cells (A549 and H1299) impaired cell proliferation, colony formation, induced cell cycle arrest and apoptosis in vitro, and inhibited tumorigenesis in a xenograft mouse model. Transcriptome analysis identified the CASP8-CDKN1A axis as potentially critical, consistent with GMDS acting upstream of apoptotic signaling in this cancer context. Lentiviral shRNA knockdown, cell viability/proliferation assays, flow cytometry (cell cycle and apoptosis), xenograft mouse model, microarray transcriptome analysis BMC cancer Medium 29843634
2025 A point mutation in mouse Gmds (ENU-induced) caused a near-complete block in T cell development at the double-positive stage in the thymus, with a cell-intrinsic requirement demonstrated by bone marrow reconstitution and mixed chimera experiments showing Gmds-deficient T cells could not compete from the DP stage onward, while B cell subsets were broadly normal. ENU mutagenesis mouse model, flow cytometry immunophenotyping, bone marrow reconstitution (Rag1 mice), mixed chimera experiments Frontiers in immunology High 40642090
2025 Loss of gmds function in zebrafish increased hair cell number in neuromasts and accelerated hair cell regeneration after neomycin-induced ablation. Pharmacological inhibition of Notch signaling enhanced hair cell regeneration in wild-type siblings but less so in gmds mutants, indicating that Notch signaling acts partially downstream of gmds in regulating hair cell regeneration. CRISPR/Cas9 gmds mutant zebrafish, neomycin hair cell ablation assay, pharmacological Notch inhibition, live imaging of hair cell regeneration International journal of molecular sciences Medium 41097001
2025 gmds haploinsufficiency in zebrafish (CRISPR/Cas9) caused retinal ganglion cell (RGC) layer thinning, RGC loss, and reduced optic nerve head width. RNA-seq showed significant downregulation of stress response genes (including crystallin family) and increased expression of cell death genes in gmds heterozygous mutant eyes, suggesting GMDS regulates ocular stress responses and RGC survival. CRISPR/Cas9 zebrafish mutant, RNAseq transcriptome analysis, histological/morphological phenotyping of eye Experimental eye research Medium 40571142

Source papers

Stage 0 corpus · 27 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2004 GMD@CSB.DB: the Golm Metabolome Database. Bioinformatics (Oxford, England) 952 15613389
2007 Double knockdown of alpha1,6-fucosyltransferase (FUT8) and GDP-mannose 4,6-dehydratase (GMD) in antibody-producing cells: a new strategy for generating fully non-fucosylated therapeutic antibodies with enhanced ADCC. BMC biotechnology 114 18047682
2007 Establishment of a GDP-mannose 4,6-dehydratase (GMD) knockout host cell line: a new strategy for generating completely non-fucosylated recombinant therapeutics. Journal of biotechnology 93 17559959
2009 Deficiency of GMDS leads to escape from NK cell-mediated tumor surveillance through modulation of TRAIL signaling. Gastroenterology 87 19361506
2019 LncRNA GMDS-AS1 inhibits lung adenocarcinoma development by regulating miR-96-5p/CYLD signaling. Cancer medicine 52 31860169
2009 The structural basis for catalytic function of GMD and RMD, two closely related enzymes from the GDP-D-rhamnose biosynthesis pathway. The FEBS journal 41 19459932
2011 GDP-mannose-4,6-dehydratase (GMDS) deficiency renders colon cancer cells resistant to tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) receptor- and CD95-mediated apoptosis by inhibiting complex II formation. The Journal of biological chemistry 39 22027835
2018 GMDS knockdown impairs cell proliferation and survival in human lung adenocarcinoma. BMC cancer 26 29843634
2001 Cloning and expression of Helicobacter pylori GDP-l-fucose synthesizing enzymes (GMD and GMER) in Saccharomyces cerevisiae. European journal of biochemistry 22 11737200
2016 Correlations of AFAP1, GMDS and PTGFR gene polymorphisms with intra-ocular pressure response to latanoprost in patients with primary open-angle glaucoma. Journal of clinical pharmacy and therapeutics 18 27862086
2024 Decitabine in older patients with AML: quality of life results of the EORTC-GIMEMA-GMDS-SG randomized phase 3 trial. Blood 16 38717861
2012 GMD: measuring the distance between histograms with applications on high-throughput sequencing reads. Bioinformatics (Oxford, England) 14 22345619
2022 lncRNA GMDS‑AS1 upregulates IL‑6, TNF‑α and IL‑1β, and induces apoptosis in human monocytic THP‑1 cells via miR‑96‑5p/caspase 2 signaling. Molecular medicine reports 13 34981821
2015 Disruptions in a cluster of computationally identified enhancers near FOXC1 and GMDS may influence brain development. Neurogenetics 12 26382291
2013 Human GMDS gene fragment hypermethylation in chronic high level of arsenic exposure with and without arsenic induced cancer. SpringerPlus 12 24255851
2023 lncRNA GMDS-AS1 restrains lung adenocarcinoma progression via recruiting TAF15 protein to stabilize SIRT1 mRNA. Epigenomics 10 37309595
2020 Generation of FX-/- and Gmds-/- CHOZN host cell lines for the production of afucosylated therapeutic antibodies. Biotechnology progress 10 32748555
2019 Gastric mucosal devitalization (GMD): translation to a novel endoscopic metabolic therapy. Endoscopy international open 9 31788546
2023 Establishment and Application of an Indirect ELISA for the Detection of Antibodies to Porcine Streptococcus suis Based on a Recombinant GMD Protein. Animals : an open access journal from MDPI 8 36830506
2023 Epigenetic inhibition of lncRNA GMDS-AS1 by methyltransferase ESET promoted cell viability and metastasis of hepatocellular carcinoma. Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico 4 36737533
2011 The balance between GMD and OFUT1 regulates Notch signaling pathway activity by modulating Notch stability. Biological research 4 21720678
2021 Interactions between the Prophage 919TP and Its Vibrio cholerae Host: Implications of gmd Mutation for Phage Resistance, Cell Auto-Aggregation, and Motility. Viruses 3 34960610
2025 Genomic analysis of glaucoma pathogenesis due to gmds mutation in zebrafish. Experimental eye research 2 40571142
2024 The Indispensable Roles of GMDS and GMDS-AS1 in the Advancement of Cancer: Fucosylation, Signal Pathway and Molecular Pathogenesis. Mini reviews in medicinal chemistry 1 38591197
2026 ROS, identified as a driver for fibroblast activation, can be counteracted by glucocorticoid in LINC00605-dependent GMD machinery. Free radical biology & medicine 0 42097320
2025 Essential cell-intrinsic requirement for GMDS in T cell development. Frontiers in immunology 0 40642090
2025 Mutation of the GDP-Fucose Biosynthesis Gene gmds Increases Hair Cell Number and Neuromast Regenerative Capacity in Zebrafish. International journal of molecular sciences 0 41097001

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