About: Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders

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description	article (en) հոդված (hy) wetenschappelijk artikel (nl) article scientifique publié en 2019 (fr) im Juni 2019 veröffentlichter wissenschaftlicher Artikel (de) наукова стаття, опублікована в червні 2019 (uk)
publication date	wds:Q64375165-BF726D09-2312-4E5A-9362-864CEC60BC48
publication date	2019-01-11 00:00:00Z (xsd:dateTime)
language of work or name	wds:Q64375165-8E81D1FA-E94C-4184-A397-47CBFAB3DC26
language of work or name	English
cites work	wds:Q64375165-03BCD536-B369-4F9E-970E-634412283A19 wds:Q64375165-11A929DF-C0AD-4A8A-B9D0-94EBE894A51D wds:Q64375165-2B2E2E3F-0CCC-44FF-B336-23720DC4BD4F wds:Q64375165-2B90D3A5-C3C2-4EF6-B5A1-0FE30447FD0E wds:Q64375165-2BFB2048-8970-4CFA-B3A4-D9B951B625F4 wds:Q64375165-2FD3EBA3-B573-4FB2-B794-80C8279A4173 wds:Q64375165-36C15E6B-1360-4622-A8D1-EB2B77A380E2 wds:Q64375165-3C2D7A1A-EE47-4564-8541-7E66757999AB wds:Q64375165-3D9860DF-F6FA-46B3-89D7-B80C2810640C wds:Q64375165-42B45A3B-9F60-479D-AB7C-E5FCB5EBACCC wds:Q64375165-4EE84ED7-4AB5-4861-8919-213EED2A570A wds:Q64375165-4FE8CDF5-F455-442B-8D75-039E80DDECD4 wds:Q64375165-51D6B8C5-E293-49CC-A146-EB19D8137B63 wds:Q64375165-55E75387-DA59-4FCB-BA80-3A4CB248FF14 wds:Q64375165-5A0D4508-DE73-47FA-8A80-8F80F11F2694 wds:Q64375165-5A61AA23-7AB1-4E19-A1D8-16FD7F85E16D wds:Q64375165-5A6CCA47-E780-45A3-839C-CA93E1368A87 wds:Q64375165-5F976303-902C-4877-AB9C-C59A4C63C6C4 wds:Q64375165-74758E12-4979-4806-BE5A-D29C55514253 wds:Q64375165-784A4185-DBA3-4526-8FD1-BC9CCDB6C3B5 wds:Q64375165-7FFCEA4F-6C01-48C2-A780-984F2D93E7E7 wds:Q64375165-95F01A5D-7FE1-43A4-9709-5F659EBD2AF8 wds:Q64375165-9B476A17-9B22-4CB4-B9E4-2139CFC2A540 wds:Q64375165-A483D94D-8558-4596-A772-C7A1F34DED66 wds:Q64375165-A5AA9434-C3D3-416E-BA97-7F566C6C2113 wds:Q64375165-BA98EDBE-170C-44FD-8B73-D1BCEFB69CD4 wds:Q64375165-BB689065-A373-4A1C-B46F-8326CC707FAC wds:Q64375165-BC8AA8EF-99EC-4FDC-87C0-8579FDECDADC wds:Q64375165-BCB25311-95DC-4190-88E0-B23C8FA8A6BE wds:Q64375165-E30F4404-3337-4DB6-A2D1-2C2964335B6C wds:Q64375165-EAFCC753-63FE-45B0-B786-0DC7ACD05FA9 wds:Q64375165-F55771C8-E6F0-408A-B876-1FBCEA78D3EE wds:Q64375165-FA5B2248-328B-4A8C-8FAC-B62D5D7C874D wds:Q64375165-FD6DABA9-B686-4500-A8EA-56A23EEC4E4E
cites work	Ectopic calcification: gathering hard facts about soft tissue mineralization Adenovirus: the first effective in vivo gene delivery vector Analysis of pseudoxanthoma elasticum-causing missense mutants of ABCC6 in vivo; pharmacological correction of the mislocalized proteins Parabiotic heterogenetic pairing of Abcc6-/-/Rag1-/- mice and their wild-type counterparts halts ectopic mineralization in a murine model of pseudoxanthoma elasticum Targeted ablation of the abcc6 gene results in ectopic mineralization of connective tissues Pseudoxanthoma elasticum is a metabolic disease Pseudoxanthoma elasticum Pseudoxanthoma elasticum: mutations in the MRP6 gene encoding a transmembrane ATP-binding cassette (ABC) transporter Mutations in a gene encoding an ABC transporter cause pseudoxanthoma elasticum Mutations in ABCC6 cause pseudoxanthoma elasticum MOAT-E (ARA) is a full-length MRP/cMOAT subfamily transporter expressed in kidney and liver Generalized arterial calcification of infancy and pseudoxanthoma elasticum can be caused by mutations in either ENPP1 or ABCC6 ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release MRP6 (ABCC6) detection in normal human tissues and tumors. ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report. Progress and problems with the use of viral vectors for gene therapy Premature termination codon read-through in the ABCC6 gene: potential treatment for pseudoxanthoma elasticum Adenoviral vectors: a promising tool for gene therapy Isolation and characterization of canalicular and basolateral plasma membrane fractions from human liver Genetic heterogeneity of pseudoxanthoma elasticum: the Chinese signature profile of ABCC6 and ENPP1 mutations Mutations in the ABCC6 gene as a cause of generalized arterial calcification of infancy: genotypic overlap with pseudoxanthoma elasticum Oral administration of pyrophosphate inhibits connective tissue calcification. Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice. Gene therapy clinical trials worldwide to 2017: An update Functional Rescue of ABCC6 Deficiency by 4-Phenylbutyrate Therapy Reduces Dystrophic Calcification in Abcc6-/- Mice. Adenovirus-Mediated Gene Delivery: Potential Applications for Gene and Cell-Based Therapies in the New Era of Personalized Medicine Gene therapy for monogenic liver diseases: clinical successes, current challenges and future prospects. Inhibition of Tissue-Nonspecific Alkaline Phosphatase Attenuates Ectopic Mineralization in the Abcc6-/- Mouse Model of PXE but Not in the Enpp1 Mutant Mouse Models of GACI Plasma PPi Deficiency Is the Major, but Not the Exclusive, Cause of Ectopic Mineralization in an Abcc6-/- Mouse Model of PXE. Etidronate prevents dystrophic cardiac calcification by inhibiting macrophage aggregation. Utilization of adenovirus vectors for multiple gene transfer applications The ABCC6 Transporter: A New Player in Biomineralization. Insights into Pathomechanisms and Treatment Development in Heritable Ectopic Mineralization Disorders: Summary of the PXE International Biennial Research Symposium-2016. Abcc6 Knockout Rat Model Highlights the Role of Liver in PPi Homeostasis in Pseudoxanthoma Elasticum.
author name string	wds:Q64375165-08111ACC-5008-4DEB-8536-491B1764719A wds:Q64375165-3249A91A-FCFB-4C66-8A75-264BDF04FB55 wds:Q64375165-5B4DB59D-2B24-4286-8D93-AFDC75063FA6
author name string	Jouni Uitto Jianhe Huang Qiaoli Li
rdfs:label	Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (en) Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (nl)
skos:prefLabel	Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (en) Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (nl)
name	Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (en) Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (nl)
author	wds:Q64375165-68A63175-CBE6-4D95-ADB5-9E799867BAF9
author	Adam E. Snook
title	wds:Q64375165-A470E47C-F911-421D-ABAA-04BA46398790
title	Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders (en)
page(s)	wds:Q64375165-9697CADE-34A7-4B47-A46A-B79448891947
page(s)	1254-1263
instance of	wds:Q64375165-2D21304F-19FC-48C3-815A-FB1004E0BA85
instance of	scholarly article
main subject	wds:Q64375165-9C17C0AC-634B-45CE-8E11-4792166EE6B4

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