Affinage

RDH10

Retinol dehydrogenase 10 · UniProt Q8IZV5

Length
341 aa
Mass
38.1 kDa
Annotated
2026-06-10
19 papers in source corpus 15 papers cited in narrative 15 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

RDH10 is a membrane-associated short-chain dehydrogenase that catalyzes the first oxidative step of vitamin A metabolism, converting retinol to retinaldehyde and thereby driving retinoic acid (RA) synthesis required across embryonic and postnatal development (PMID:17473173, PMID:18502750). Biochemically it is strictly NAD+-dependent with an exceptionally low Km for all-trans-retinol (~0.035 µM), acts exclusively in the oxidative direction in cells, and is the rate-limiting source of retinaldehyde feeding RA production (PMID:18502750); this activity is partitioned into a membrane-bound compartment that shields the reaction from inhibition by cytosolic CRBP1, making cytosolic RDH activity dispensable under normal conditions (PMID:21782811). The enzyme relocates dynamically between mitochondria/mitochondrial-associated membranes and lipid droplets depending on acyl ester biosynthesis, with distinct N- and C-terminal hydrophobic determinants governing each targeting outcome (PMID:23155051). In the retina, RDH10 additionally oxidizes 11-cis-retinol within a reconstituted visual cycle and physically interacts with CRALBP and RPE65 (PMID:19458327). Loss-of-function genetics establish RDH10 as the principal driver of embryonic RA signaling: the trex missense allele abolishes activity and produces craniofacial, limb, and organ defects (PMID:17473173), and tissue- and stage-specific inactivation shows RA generated by RDH10 controls interdigital regression (PMID:21360789), choanae formation (PMID:28169399), salivary gland initiation through RARα and SOX9 (PMID:29986869), palate closure via fetal mouth movement (PMID:31300413), and optic cup formation through the direct RA target Alx1 [PMID:bio_10.1101_2025.06.24.661406]. Postnatally, RDH10 haploinsufficiency lowers atRA in liver and adipose tissue, escalating adipogenesis and producing adiposity, steatosis, and insulin resistance reversible by atRA (PMID:29321172). In dendritic cells RDH10 operates within a PPARγ-regulated RDH10–RALDH2–CRABP2 pathway for ATRA production (PMID:23833249).

Mechanistic history

Synthesis pass · year-by-year structured walk · 15 steps
  1. 2004 Medium

    First localized RDH10's retinol dehydrogenase activity to microsomal membranes in retinal cells, establishing it as a generator of all-trans retinal feeding downstream photoisomerase activity.

    Evidence IHC, RT-PCR and HPLC-based RDH activity assays on microsomal fractions of Müller (rMC-1) cells

    PMID:15505029

    Open questions at the time
    • Reported NADP preference later overturned for the human enzyme
    • Single-lab study without genetic loss-of-function
  2. 2007 High

    Defined RDH10 as the enzyme catalyzing the first oxidative step of vitamin A metabolism and demonstrated its requirement for embryonic RA signaling via a loss-of-function allele.

    Evidence ENU forward genetic screen yielding the trex missense allele, enzymatic assays, and mutant embryo phenotyping

    PMID:17473173

    Open questions at the time
    • Cofactor identity not yet resolved at this stage
    • Subcellular site of catalysis not defined
  3. 2007 Low

    Linked RDH10-driven RA production to growth control by showing overexpression raises RA and arrests hepatocellular carcinoma cell proliferation.

    Evidence Stable RDH10 overexpression in HepG2 cells with RARE-CAT reporter, proliferation assays, and cell-cycle gene RT-PCR

    PMID:17218779

    Open questions at the time
    • Single overexpression experiment with indirect RA readout
    • No endogenous loss-of-function in tumor context
    • Mechanism of antiproliferative effect inferred only from mRNA changes
  4. 2008 High

    Resolved the enzymology: RDH10 is strictly NAD+-dependent with very high affinity for all-trans-retinol and functions only in the oxidative direction, correcting the earlier NADP+ assignment.

    Evidence Kinetic assays with purified recombinant enzyme, cofactor specificity tests, and siRNA knockdown with retinoid quantification in human cells

    PMID:18502750

    Open questions at the time
    • No crystal structure or active-site model
    • Membrane topology not defined
  5. 2009 High

    Extended RDH10's role into the visual cycle, showing it oxidizes 11-cis-retinol and physically partners with CRALBP and RPE65.

    Evidence In vitro 11-cis-RDH assay, reconstituted visual cycle in HEK-293A cells, co-immunoprecipitation, and IHC in bovine RPE

    PMID:19458327

    Open questions at the time
    • Physiological contribution to the visual cycle in vivo not established
    • Co-IP not reciprocally validated for direct binding
  6. 2011 High

    Established that the rate-limiting retinol oxidation occurs in a membrane compartment, explaining why the reaction escapes CRBP1 inhibition and why cytosolic RDHs are dispensable.

    Evidence Rdh10trex embryos, membrane vs cytosolic RDH activity assays, and dietary retinaldehyde rescue

    PMID:21782811

    Open questions at the time
    • Exact membrane identity not pinned in this study
    • Does not address tissue-specific enzyme redundancy
  7. 2011 High

    Dissected the developmental specificity of RDH10-dependent RA, showing it drives interdigital regression but is not required for limb skeletal patterning.

    Evidence RARE-lacZ reporter, exogenous RA rescue, skeletal staining and in situ hybridization in Rdh10trex mutants

    PMID:21360789

    Open questions at the time
    • Source of residual patterning RA in mutant limb unresolved
    • Cellular mechanism of interdigital cell loss not detailed
  8. 2012 Medium

    Revealed dynamic subcellular partitioning of RDH10 between mitochondria/MAM and lipid droplets and mapped the N- and C-terminal determinants of targeting.

    Evidence Subcellular fractionation, domain deletion mutants, and fluorescence colocalization with CRBP1 and LRAT

    PMID:23155051

    Open questions at the time
    • Proposed retinol metabolon not biochemically reconstituted
    • Functional consequence of relocation for RA output not measured
    • Single-lab cell biology study
  9. 2013 Medium

    Placed RDH10 within a defined PPARγ-regulated linear ATRA-synthesis pathway in dendritic cells alongside RALDH2 and CRABP2.

    Evidence siRNA knockdown of RDH10, RALDH2 and CRABP2 in human mo-DCs with ATRA measurement and PPARγ activation assays

    PMID:23833249

    Open questions at the time
    • Direct transcriptional regulation of RDH10 by PPARγ not shown at promoter level
    • In vivo immune consequence not tested
  10. 2017 Medium

    Defined a cell-type- and stage-specific requirement for RDH10 in non-neural-crest cells for choanae formation, linking RA loss to ectopic Fgf8 and epithelial defects.

    Evidence Conditional/temporal Rdh10 mutants, lineage tracing, Fgf8 in situ, and proliferation/apoptosis assays

    PMID:28169399

    Open questions at the time
    • Direct RA target genes in nasal epithelium not identified
    • Single-lab study
  11. 2018 High

    Demonstrated a postnatal metabolic role: RDH10 haploinsufficiency lowers atRA and escalates adipogenesis, adiposity and insulin resistance, reversible by atRA.

    Evidence Rdh10 heterozygote and knockout mice, LC-MS atRA quantification, adipogenesis assays, metabolic phenotyping, and pharmacological rescue

    PMID:29321172

    Open questions at the time
    • Tissue-autonomous vs systemic contribution not fully separated
    • RAR target genes in adipocytes not enumerated
  12. 2018 Medium

    Showed RDH10-derived RA acts specifically through RARα to drive SOX9 expression and epithelial invagination in salivary gland initiation.

    Evidence Ex vivo salivary gland initiation assay, stage-specific Rdh10 inactivation, and RAR isoform-specific inhibitors/agonists

    PMID:29986869

    Open questions at the time
    • Direct RARα target genes upstream of SOX9 not defined
    • Single-lab ex vivo system
  13. 2019 Medium

    Uncovered a mechanical mechanism for RDH10-dependent palate closure, where RA-dependent pharyngeal patterning enables fetal mouth movement required for shelf elevation.

    Evidence Stage-specific Rdh10 inactivation, X-ray microtomography, in utero ultrasound, and tissue staining of nerves and skeletal elements

    PMID:31300413

    Open questions at the time
    • Molecular RA targets in pharyngeal nerve/skeletal patterning not identified
    • Single-lab study
  14. 2025 Medium

    Identified Alx1 as a direct RA target downstream of RDH10 in optic cup formation, providing a molecular effector for an RDH10-dependent developmental phenotype.

    Evidence Rdh10 KO mouse with H3K27ac ChIP-seq, RNA-seq, RARE mapping, and CRISPR Alx1 knockout phenocopy (preprint)

    PMID:bio_10.1101_2025.06.24.661406

    Open questions at the time
    • Preprint not yet peer-reviewed
    • Direct RARE occupancy not validated by reporter assay
  15. 2025 Medium

    Established a requirement for RDH10 in the foregut mesenchymal microenvironment for vagal neural crest invasion, linking RA loss to Ret-Gdnf-Gfrα1 signaling and ECM changes underlying enteric aganglionosis.

    Evidence Stage-specific Rdh10 loss-of-function mice, NCC lineage tracing, RNA-seq, and ECM analysis (preprint)

    PMID:39896510

    Open questions at the time
    • Preprint not yet peer-reviewed
    • Direct RA-responsive elements in Ret-Gdnf network not mapped
    • Causality of ECM changes vs signaling not separated

Open questions

Synthesis pass · forward-looking unresolved questions
  • How RDH10's dynamic membrane localization, cofactor handling, and physical interactions are mechanistically coupled to control RA output in different tissues remains unresolved.
  • No structural model of the enzyme or its membrane topology
  • The proposed lipid-droplet retinol metabolon is not biochemically reconstituted
  • Tissue-specific partner sets directing RDH10 output are uncharacterized

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016491 oxidoreductase activity 3
Localization
GO:0005783 endoplasmic reticulum 2 GO:0005739 mitochondrion 1 GO:0005811 lipid droplet 1
Pathway
R-HSA-1266738 Developmental Biology 5 R-HSA-1430728 Metabolism 3 R-HSA-9709957 Sensory Perception 1

Evidence

Reading pass · 15 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2007 RDH10 catalyzes the first oxidative step of vitamin A metabolism — the oxidation of retinol to retinal — and is required for embryonic retinoic acid synthesis. A missense mutation in RDH10 (trex allele) abolishes this retinol dehydrogenase activity, resulting in insufficient RA signaling and craniofacial, limb, and organ defects. ENU forward genetic screen, protein modeling, enzymatic activity assays, and analysis of mutant embryos Genes & development High 17473173
2004 RDH10 is expressed in retinal Müller cells (in addition to RPE) and its all-trans retinol dehydrogenase activity localizes to the microsomal fraction, using NADP as a preferred cofactor in those cells. It generates all-trans retinal, which serves as substrate for the photoisomerase RGR in Müller cells. Western blot, immunohistochemistry, RT-PCR, HPLC-based retinol dehydrogenase activity assay on microsomal fractions of rMC-1 cells Investigative ophthalmology & visual science Medium 15505029
2008 Human RDH10 is a strictly NAD+-dependent enzyme (not NADP+-dependent as initially reported) with multisubstrate specificity, recognizing both all-trans-retinol and cis-retinols as substrates. It has an exceptionally low apparent Km for all-trans-retinol (~0.035 µM) but a relatively high Km for NAD+ (~100 µM). RDH10 functions exclusively in the oxidative direction in cells, increasing retinaldehyde and retinoic acid levels. siRNA-mediated knockdown of endogenous RDH10 in human cells significantly decreases retinoic acid production from retinol. Kinetic enzymatic assays with purified recombinant enzyme, cofactor specificity assays, siRNA knockdown with retinoid quantification The Journal of biological chemistry High 18502750
2009 RDH10 oxidizes 11-cis-retinol to 11-cis-retinaldehyde in vitro (11-cis-RDH activity), stimulated by CRALBP. In a reconstituted visual cycle cell culture model (RDH10 + RPE65 + LRAT + CRALBP co-expression), 11-cis-retinaldehyde is generated from all-trans-retinol. RDH10 physically interacts with CRALBP and RPE65 by co-immunoprecipitation and co-localizes with them in bovine RPE cells. In vitro 11-cis-RDH activity assay in COS1 cells, reconstituted visual cycle in HEK-293A cells, co-immunoprecipitation, immunohistochemistry, HPLC retinoid profiling Investigative ophthalmology & visual science High 19458327
2011 RDH10 is the primary retinol dehydrogenase responsible for the first oxidative step of embryonic vitamin A metabolism. The initial retinol-to-retinal conversion occurs predominantly in a membrane-bound cellular compartment, which prevents inhibition by cytosolic CRBP1 (RBP1). Cytosolic enzymes with RDH activity play a very limited role under normal dietary conditions. Rdh10trex mutant embryos, dietary retinaldehyde supplementation, RDH activity assays on membrane vs. cytosolic fractions Developmental biology High 21782811
2011 RDH10 (via RA synthesis) is required for interdigital tissue loss but not for limb patterning per se. In Rdh10trex/trex mutants, RA activity is absent from limb mesoderm but present in neuroectoderm; restoration with 25 nM RA rescues RARE-lacZ activity in limb mesoderm. Meis2 and Shh expression and skeletal patterning are normal in Rdh10 mutant hindlimbs despite absent limb RA. RARE-lacZ RA-reporter transgene, exogenous RA rescue, skeletal staining, in situ hybridization in Rdh10trex/trex mutant embryos Developmental dynamics High 21360789
2012 Rdh10 associates predominantly with mitochondria/mitochondrial-associated membrane (MAM) in the absence of lipid droplet biosynthesis, but relocates to lipid droplets during acyl ester biosynthesis. The 32 N-terminal residues (including a hydrophobic region followed by net positive charge) are required for lipid droplet targeting; both N-terminal and 48 C-terminal hydrophobic residues are required for mitochondria/MAM targeting and/or protein stability. Co-localization of Rdh10, CRBP1, and LRAT on lipid droplets suggests a metabolon for retinol homeostasis. Subcellular fractionation, domain deletion mutants, fluorescence colocalization, cell biology assays The Journal of biological chemistry Medium 23155051
2013 In human monocyte-derived dendritic cells, RDH10, RALDH2, and CRABP2 form a linear PPARγ-regulated pathway required for ATRA production. All three proteins are co-regulated by PPARγ activation and all three are required for ATRA synthesis induced by PPARγ-activating fatty acids. siRNA knockdown of RDH10, RALDH2, and CRABP2 in human mo-DCs with ATRA measurement; PPARγ activation assays; colocalization in gut-associated lymphoid tissue DCs Journal of lipid research Medium 23833249
2017 Rdh10 is specifically required in non-neural crest cells prior to E10.5 for proper choanae formation. Loss of Rdh10 leads to ectopic Fgf8 expression in the nasal fin, decreased cell proliferation, and increased cell death in the nasal cavity epithelium, retarding invagination and causing fully penetrant choanal atresia. Conditional/temporal Rdh10 mutant mouse analysis, cell lineage tracing, in situ hybridization for Fgf8, cell proliferation and apoptosis assays Human molecular genetics Medium 28169399
2018 Rdh10 heterozygous hypomorphs produce ~25% less atRA in liver and adipose tissue, leading to escalated adipogenesis, increased adiposity under high-fat diet, liver steatosis, glucose intolerance, and insulin resistance. Embryonic fibroblasts with Rdh10 knockout show decreased atRA biosynthesis and escalated adipogenesis reversible by atRA or RAR pan-agonist treatment. Rdh10 heterozygote and knockout mouse models, atRA quantification by LC-MS, adipogenesis assays, metabolic phenotyping, pharmacological rescue with atRA Diabetes High 29321172
2018 RDH10-mediated retinol metabolism and RARα-mediated RA signaling are required for submandibular salivary gland initiation. RDH10 and RALDH2 are expressed in the SMG mesenchyme at the initiation site, and ex vivo assays demonstrate that RDH10 and RA are both required for SOX9 expression and epithelial invagination. The RA requirement acts specifically through RARα with no contribution from other RAR isoforms. Ex vivo salivary gland initiation assay, stage-specific Rdh10 inactivation, RAR isoform-specific inhibitors/agonists, in situ hybridization Development (Cambridge, England) Medium 29986869
2019 RDH10 function (via RA synthesis) is required for spontaneous fetal mouth movement that facilitates palate shelf elevation. Rdh10-deficient embryos display mispatterned pharyngeal nerves and skeletal elements that physically block fetal mouth movement in utero, causing cleft palate through a mechanical (movement-dependent) mechanism rather than a direct tissue defect in the palate shelf. Stage-specific Rdh10 inactivation, X-ray microtomography, in utero ultrasound video, ex vivo culture, tissue staining of pharyngeal nerves and skeletal elements Disease models & mechanisms Medium 31300413
2007 Forced over-expression of RDH10 in HepG2 hepatocellular carcinoma cells increases endogenous RA concentration (measured by RARE-CAT reporter), causes antiproliferative effects without apoptosis, and is associated with upregulation of RARβ and p21Cip1 and downregulation of CyclinE/CDK2 mRNAs. Stable RDH10 over-expression in HepG2 cells, RARE-CAT reporter assay, RT-PCR for cell cycle gene expression, proliferation assays Cancer biology & therapy Low 17218779
2025 Rdh10 knockout embryos fail to form a proper optic cup. Combined ChIP-seq (H3K27ac) and RNA-seq on eye tissue identified Alx1 as a direct RA target gene with an RA response element (RARE) near an RA-regulated H3K27ac mark. CRISPR/Cas9 knockout of Alx1 phenocopies Rdh10 KO in optic cup formation, placing Alx1 downstream of RDH10-mediated RA synthesis in eye development. Rdh10 knockout mouse, ChIP-seq for H3K27ac, RNA-seq on eye tissue, CRISPR/Cas9 Alx1 knockout, in situ hybridization bioRxivpreprint Medium bio_10.1101_2025.06.24.661406
2025 Rdh10 is highly expressed in the mesenchyme surrounding the entrance to the foregut and is essential between E7.5–E9.5 for vagal neural crest cell invasion into the gut. Rdh10 loss-of-function embryos exhibit intestinal aganglionosis; NCC form and migrate normally but fail to invade the foregut. RNA-seq revealed downregulation of the Ret-Gdnf-Gfrα1 signaling network and altered extracellular matrix (increased collagen deposition) in the NCC microenvironment. Rdh10 loss-of-function mouse, stage-specific inactivation (E7.5–E9.5), NCC lineage tracing, comparative RNA-seq, extracellular matrix analysis bioRxivpreprint Medium 39896510

Source papers

Stage 0 corpus · 19 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2007 RDH10 is essential for synthesis of embryonic retinoic acid and is required for limb, craniofacial, and organ development. Genes & development 276 17473173
2011 RDH10 is the primary enzyme responsible for the first step of embryonic Vitamin A metabolism and retinoic acid synthesis. Developmental biology 63 21782811
2004 Identification of RDH10, an All-trans Retinol Dehydrogenase, in Retinal Muller Cells. Investigative ophthalmology & visual science 60 15505029
2009 The 11-cis-retinol dehydrogenase activity of RDH10 and its interaction with visual cycle proteins. Investigative ophthalmology & visual science 57 19458327
2008 Kinetic analysis of human enzyme RDH10 defines the characteristics of a physiologically relevant retinol dehydrogenase. The Journal of biological chemistry 57 18502750
2011 Rdh10 mutants deficient in limb field retinoic acid signaling exhibit normal limb patterning but display interdigital webbing. Developmental dynamics : an official publication of the American Association of Anatomists 53 21360789
2007 Expression of the murine retinol dehydrogenase 10 (Rdh10) gene correlates with many sites of retinoid signalling during embryogenesis and organ differentiation. Developmental dynamics : an official publication of the American Association of Anatomists 48 17849458
2018 Modest Decreases in Endogenous All-trans-Retinoic Acid Produced by a Mouse Rdh10 Heterozygote Provoke Major Abnormalities in Adipogenesis and Lipid Metabolism. Diabetes 42 29321172
2012 The retinol dehydrogenase Rdh10 localizes to lipid droplets during acyl ester biosynthesis. The Journal of biological chemistry 41 23155051
2012 Morphological defects in a novel Rdh10 mutant that has reduced retinoic acid biosynthesis and signaling. Genesis (New York, N.Y. : 2000) 36 22162152
2008 Dynamic expression of the retinoic acid-synthesizing enzyme retinol dehydrogenase 10 (rdh10) in the developing mouse brain and sensory organs. The Journal of comparative neurology 32 18399539
2017 Rdh10 loss-of-function and perturbed retinoid signaling underlies the etiology of choanal atresia. Human molecular genetics 26 28169399
2013 RDH10, RALDH2, and CRABP2 are required components of PPARγ-directed ATRA synthesis and signaling in human dendritic cells. Journal of lipid research 24 23833249
2010 The expression of Stra6 and Rdh10 in the avian embryo and their contribution to the generation of retinoid signatures. The International journal of developmental biology 20 20563989
2018 RDH10-mediated retinol metabolism and RARα-mediated retinoic acid signaling are required for submandibular salivary gland initiation. Development (Cambridge, England) 19 29986869
2007 Forced expression of RDH10 gene retards growth of HepG2 cells. Cancer biology & therapy 15 17218779
2019 RDH10 function is necessary for spontaneous fetal mouth movement that facilitates palate shelf elevation. Disease models & mechanisms 14 31300413
2003 Genomic organization and transcription of the human retinol dehydrogenase 10 (RDH10) gene. FEBS letters 9 14596915
2025 Rdh10-mediated Retinoic Acid Signaling Regulates the Neural Crest Cell Microenvironment During ENS Formation. bioRxiv : the preprint server for biology 2 39896510

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