| scholarly article | Q13442814 |
| P356 | DOI | 10.1016/J.YMGME.2019.01.007 |
| P698 | PubMed publication ID | 30737139 |
| P50 | author | Robert J. Desnick | Q7345862 |
| P2093 | author name string | Makiko Yasuda | |
| P2860 | cites work | Erythropoietic protoporphyria | Q21202891 |
| Effective gene therapy of mice with congenital erythropoietic porphyria is facilitated by a survival advantage of corrected erythroid cells | Q24644026 | ||
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| Erythropoietic protoporphyria | Q28242869 | ||
| Harderoporphyria due to homozygosity for coproporphyrinogen oxidase missense mutation H327R | Q28299154 | ||
| Homozygous variegate porphyria: an evolving clinical syndrome | Q28384495 | ||
| A mouse model of familial porphyria cutanea tarda | Q28507743 | ||
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| Increased plasma transferrin, altered body iron distribution, and microcytic hypochromic anemia in ferrochelatase-deficient mice | Q28590826 | ||
| Ferrochelatase structural mutant (Fechm1Pas) in the house mouse | Q28592856 | ||
| Heme-regulated eIF2alpha kinase modifies the phenotypic severity of murine models of erythropoietic protoporphyria and beta-thalassemia | Q28592905 | ||
| An exon 10 deletion in the mouse ferrochelatase gene has a dominant-negative effect and causes mild protoporphyria | Q28593649 | ||
| Reversion of hepatobiliary alterations By bone marrow transplantation in a murine model of erythropoietic protoporphyria | Q33180600 | ||
| A knock-in mouse model of congenital erythropoietic porphyria. | Q33228017 | ||
| Feline acute intermittent porphyria: a phenocopy masquerading as an erythropoietic porphyria due to dominant and recessive hydroxymethylbilane synthase mutations | Q33594209 | ||
| RNAi-mediated silencing of hepatic Alas1 effectively prevents and treats the induced acute attacks in acute intermittent porphyria mice | Q33694417 | ||
| AAV8-mediated gene therapy prevents induced biochemical attacks of acute intermittent porphyria and improves neuromotor function | Q33730418 | ||
| Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria | Q33849564 | ||
| Feline congenital erythropoietic porphyria: two homozygous UROS missense mutations cause the enzyme deficiency and porphyrin accumulation | Q34112712 | ||
| Liver disease and erythropoietic protoporphyria: a concise review. | Q34139190 | ||
| Acute intermittent porphyria: studies of the severe homozygous dominant disease provides insights into the neurologic attacks in acute porphyrias | Q34366289 | ||
| Sustained enzymatic correction by rAAV-mediated liver gene therapy protects against induced motor neuropathy in acute porphyria mice. | Q34556835 | ||
| CYP1A2*1F and GSTM1 alleles are associated with susceptibility to porphyria cutanea tarda | Q34699532 | ||
| Pro-oxidant effects of δ-aminolevulinic acid (δ -ALA) on Chinese hamster ovary (CHO) cells | Q73497805 | ||
| Uroporphyria in the uroporphyrinogen decarboxylase-deficient mouse: Interplay with siderosis and polychlorinated biphenyl exposure | Q77150107 | ||
| C73R is a hotspot mutation in the uroporphyrinogen III synthase gene in congenital erythropoietic porphyria | Q77515790 | ||
| Biliary fibrosis associated with altered bile composition in a mouse model of erythropoietic protoporphyria | Q78177007 | ||
| Liver pathology and hepatocarcinogenesis in a long-term mouse model of erythropoietic protoporphyria | Q78813037 | ||
| A mouse model provides evidence that genetic background modulates anemia and liver injury in erythropoietic protoporphyria | Q81333559 | ||
| Neonatal bone marrow transplantation prevents liver disease in a murine model of erythropoietic protoporphyria | Q82824225 | ||
| Porphyrias | Q83185413 | ||
| Protoporphyrin retention in hepatocytes and Kupffer cells prevents sclerosing cholangitis in erythropoietic protoporphyria mouse model | Q84564481 | ||
| Porphyria | Q88876085 | ||
| Bioengineered PBGD variant improves the therapeutic index of gene therapy vectors for acute intermittent porphyria | Q90807444 | ||
| Homozygous hydroxymethylbilane synthase knock-in mice provide pathogenic insights into the severe neurological impairments present in human homozygous dominant acute intermittent porphyria | Q90919328 | ||
| Identification of the xenosensors regulating human 5-aminolevulinate synthase | Q34836730 | ||
| Congenital Erythropoietic Porphyria: Characterization of Murine Models of the Severe Common (C73R/C73R) and Later-Onset Genotypes | Q35136687 | ||
| Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver | Q36167051 | ||
| Modeling the ferrochelatase c.315-48C modifier mutation for erythropoietic protoporphyria (EPP) in mice. | Q36250795 | ||
| Afamelanotide for Erythropoietic Protoporphyria | Q36657737 | ||
| Preclinical Development of a Subcutaneous ALAS1 RNAi Therapeutic for Treatment of Hepatic Porphyrias Using Circulating RNA Quantification | Q36928112 | ||
| Therapeutic potential of proteasome inhibitors in congenital erythropoietic porphyria. | Q37318251 | ||
| Hemolytic anemia repressed hepcidin level without hepatocyte iron overload: lesson from Günther disease model | Q37618819 | ||
| Adenoviral-mediated expression of porphobilinogen deaminase in liver restores the metabolic defect in a mouse model of acute intermittent porphyria | Q40491304 | ||
| Mitochondrial and nuclear DNA damage induced by 5-aminolevulinic acid. | Q40492564 | ||
| Phase I open label liver-directed gene therapy clinical trial for acute intermittent porphyria | Q40741783 | ||
| Gene therapy of a mouse model of protoporphyria with a self-inactivating erythroid-specific lentiviral vector without preselection. | Q40775938 | ||
| A mouse model of hereditary coproporphyria identified in an ENU mutagenesis screen | Q41440547 | ||
| Acute porphyrias: pathogenesis of neurological manifestations | Q41727540 | ||
| Chemically-induced formation of an inhibitor of hepatic uroporphyrinogen decarboxylase in inbred mice with iron overload | Q41860271 | ||
| Mitochondrial energetic defects in muscle and brain of a Hmbs-/- mouse model of acute intermittent porphyria | Q42478276 | ||
| Mouse Model For Protoporphyria I. The Liver and Hepatic Protoporphyrin Crystals | Q43543475 | ||
| Liver transplantation as a cure for acute intermittent porphyria | Q44786720 | ||
| Immunoreactive uroporphyrinogen decarboxylase in the liver in porphyria cutanea tarda | Q44872150 | ||
| Erythropoietic protoporphyria and iron therapy | Q44917391 | ||
| In vivo oxygen radical generation in the skin of the protoporphyria model mouse with visible light exposure: an L-band ESR study | Q44919267 | ||
| Hematopoietic stem cell gene therapy of murine protoporphyria by methylguanine-DNA-methyltransferase-mediated in vivo drug selection | Q45000795 | ||
| Long-term cure of the photosensitivity of murine erythropoietic protoporphyria by preselective gene therapy | Q45859178 | ||
| An inducible promoter responsive to different porphyrinogenic stimuli improves gene therapy vectors for acute intermittent porphyria | Q45872939 | ||
| Vitamin D-binding protein as a biomarker of active disease in acute intermittent porphyria | Q45876007 | ||
| Helper-dependent adenoviral liver gene therapy protects against induced attacks and corrects protein folding stress in acute intermittent porphyria mice. | Q45886447 | ||
| Cytosolic persistence of mouse brain CYP1A1 in chronic heme deficiency | Q46819729 | ||
| Positioning of proteasome inhibitors in therapy of solid malignancies | Q47353792 | ||
| Glucose metabolism during fasting is altered in experimental porphobilinogen deaminase deficiency. | Q48882834 | ||
| Hepatocyte transplantation ameliorates the metabolic abnormality in a mouse model of acute intermittent porphyria | Q51071328 | ||
| Effects of volatile anaesthetics on heme metabolism in a murine genetic model of Acute Intermittent Porphyria. A comparative study with other porphyrinogenic drugs | Q52687373 | ||
| Porphobilinogen deaminase deficiency in mice causes a neuropathy resembling that of human hepatic porphyria | Q57083724 | ||
| Systemic messenger RNA as an etiological treatment for acute intermittent porphyria | Q57295544 | ||
| Acute intermittent porphyria causes hepatic mitochondrial energetic failure in a mouse model | Q59590280 | ||
| Liver Transplantation From Donors With Acute Intermittent Porphyria | Q61796588 | ||
| Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria | Q63884081 | ||
| Homozygous case of hereditary coproporphyria | Q66697614 | ||
| Bovine protoporphyria: the first nonhuman model of this hereditary photosensitizing disease | Q67680232 | ||
| Homozygous acute intermittent porphyria: compound heterozygosity for adjacent base transitions in the same codon of the porphobilinogen deaminase gene | Q68111538 | ||
| Erythropoietic protoporphyria exacerbated by oral iron therapy | Q68483149 | ||
| A retrospective study of a patient with homozygous form of acute intermittent porphyria | Q68613523 | ||
| Identification of the mutations in the parents of a patient with a putative compound heterozygosity for acute intermittent porphyria | Q68613527 | ||
| Iron therapy for hepatic dysfunction in erythropoietic protoporphyria | Q69537021 | ||
| Instability of hematin used in the treatment of acute hepatic porphyria | Q69554140 | ||
| Complete suppression of the symptoms of congenital erythropoietic porphyria by long-term treatment with high-level transfusions | Q70035060 | ||
| The prooxidant effect of 5-aminolevulinic acid in the brain tissue of rats: implications in neuropsychiatric manifestations in porphyrias | Q71294301 | ||
| Compound heterozygous hereditary coproporphyria with fluorescing teeth | Q73038635 | ||
| P433 | issue | 3 | |
| P304 | page(s) | 332-341 | |
| P577 | publication date | 2019-01-18 | |
| P1433 | published in | Molecular Genetics and Metabolism | Q6895949 |
| P1476 | title | Murine models of the human porphyrias: Contributions toward understanding disease pathogenesis and the development of new therapies | |
| P478 | volume | 128 |
| Q92986782 | Porphyrin-Induced Protein Oxidation and Aggregation as a Mechanism of Porphyria-Associated Cell Injury | cites work | P2860 |
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