{"id":"https://openalex.org/W86100423","doi":null,"title":"Exploration of mutations in erythroid 5-aminolevulinate synthase that lead to increased porphyrin synthesis","display_name":"Exploration of mutations in erythroid 5-aminolevulinate synthase that lead to increased porphyrin synthesis","publication_year":2014,"publication_date":"2014-01-01","ids":{"openalex":"https://openalex.org/W86100423","mag":"86100423"},"language":"en","primary_location":{"id":"pmh:oai:digitalcommons.usf.edu:etd-6217","doi":null,"is_oa":true,"landing_page_url":"https://digitalcommons.usf.edu/etd/5021","pdf_url":"https://digitalcommons.usf.edu/cgi/viewcontent.cgi?article=6217&context=etd","source":{"id":"https://openalex.org/S4377196272","display_name":"Digital Commons - University of South Florida (University of South Florida)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2613432","host_organization_name":"University of South Florida","host_organization_lineage":["https://openalex.org/I2613432"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"USF Tampa Graduate Theses and Dissertations","raw_type":"dissertation"},"volume":null,"type":"dissertation","is_traditional":true,"indexed_in":[],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://digitalcommons.usf.edu/cgi/viewcontent.cgi?article=6217&context=etd","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5057303729","display_name":"Erica J. Fratz\u2010Berilla","orcid":"https://orcid.org/0000-0002-9259-0540","observed_orcids":["https://orcid.org/0000-0002-9259-0540"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Erica J. Fratz\u2010Berilla","raw_affiliation_strings":[],"raw_orcid":"https://orcid.org/0000-0002-9259-0540","affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5057303729"],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12441","display_name":"Porphyrin Metabolism and Disorders","score":0.8964999914169312,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T12441","display_name":"Porphyrin Metabolism and Disorders","score":0.8964999914169312,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10957","display_name":"Photodynamic Therapy Research Studies","score":0.10080000013113022,"subfield":{"id":"https://openalex.org/subfields/1306","display_name":"Cancer Research"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10903","display_name":"Porphyrin and Phthalocyanine Chemistry","score":0.0015999999595806003,"subfield":{"id":"https://openalex.org/subfields/2505","display_name":"Materials Chemistry"},"field":{"id":"https://openalex.org/fields/25","display_name":"Materials Science"},"domain":{"id":"https://openalex.org/domains/3","display_name":"Physical Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/5-aminolevulinic-acid-synthase","display_name":"5-aminolevulinate synthase","score":0.9459999799728394},{"id":"https://openalex.org/keywords/alas2","display_name":"ALAS2","score":0.7630000114440918},{"id":"https://openalex.org/keywords/porphyrin-accumulation","display_name":"porphyrin accumulation","score":0.8309999704360962},{"id":"https://openalex.org/keywords/protoporphyrin-ix","display_name":"protoporphyrin IX","score":0.777999997138977},{"id":"https://openalex.org/keywords/heme-biosynthesis","display_name":"heme biosynthesis","score":0.7540000081062317},{"id":"https://openalex.org/keywords/photodynamic-therapy","display_name":"photodynamic therapy","score":0.7089999914169312},{"id":"https://openalex.org/keywords/photosensitizers","display_name":"photosensitizers","score":0.7559999823570251},{"id":"https://openalex.org/keywords/catalytic-rate","display_name":"catalytic rate","score":0.6140000224113464}],"concepts":[{"id":"https://openalex.org/C2779480358","wikidata":"https://www.wikidata.org/wiki/Q422926","display_name":"Porphyrin","level":2,"score":0.6221594214439392},{"id":"https://openalex.org/C112243037","wikidata":"https://www.wikidata.org/wiki/Q22327117","display_name":"ATP synthase","level":3,"score":0.5448139309883118},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.4827286899089813},{"id":"https://openalex.org/C501734568","wikidata":"https://www.wikidata.org/wiki/Q42918","display_name":"Mutation","level":3,"score":0.4340304136276245},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.3721737861633301},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.3571224808692932},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.344443678855896},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.2996833324432373},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.23001575469970703}],"mesh":[],"locations_count":3,"locations":[{"id":"pmh:oai:digitalcommons.usf.edu:etd-6217","doi":null,"is_oa":true,"landing_page_url":"https://digitalcommons.usf.edu/etd/5021","pdf_url":"https://digitalcommons.usf.edu/cgi/viewcontent.cgi?article=6217&context=etd","source":{"id":"https://openalex.org/S4377196272","display_name":"Digital Commons - University of South Florida (University of South Florida)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2613432","host_organization_name":"University of South Florida","host_organization_lineage":["https://openalex.org/I2613432"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"USF Tampa Graduate Theses and Dissertations","raw_type":"dissertation"},{"id":"pmh:oai:scholarcommons.usf.edu:etd-6217","doi":null,"is_oa":false,"landing_page_url":"https://scholarcommons.usf.edu/etd/5021","pdf_url":null,"source":{"id":"https://openalex.org/S4377196272","display_name":"Digital Commons - University of South Florida (University of South Florida)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2613432","host_organization_name":"University of South Florida","host_organization_lineage":["https://openalex.org/I2613432"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Graduate Theses and Dissertations","raw_type":"text"},{"id":"mag:86100423","doi":null,"is_oa":false,"landing_page_url":"https://digitalcommons.usf.edu/cgi/viewcontent.cgi?article=6217&context=etd","pdf_url":null,"source":null,"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":null}],"best_oa_location":{"id":"pmh:oai:digitalcommons.usf.edu:etd-6217","doi":null,"is_oa":true,"landing_page_url":"https://digitalcommons.usf.edu/etd/5021","pdf_url":"https://digitalcommons.usf.edu/cgi/viewcontent.cgi?article=6217&context=etd","source":{"id":"https://openalex.org/S4377196272","display_name":"Digital Commons - University of South Florida (University of South Florida)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2613432","host_organization_name":"University of South Florida","host_organization_lineage":["https://openalex.org/I2613432"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"USF Tampa Graduate Theses and Dissertations","raw_type":"dissertation"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.766700029373169}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W86100423.pdf","grobid_xml":"https://content.openalex.org/works/W86100423.grobid-xml"},"referenced_works_count":227,"referenced_works":["https://openalex.org/W17356864","https://openalex.org/W20093542","https://openalex.org/W34990569","https://openalex.org/W53301098","https://openalex.org/W60529302","https://openalex.org/W65911577","https://openalex.org/W71515697","https://openalex.org/W145554458","https://openalex.org/W149801576","https://openalex.org/W152918045","https://openalex.org/W176015410","https://openalex.org/W184040730","https://openalex.org/W203009548","https://openalex.org/W262216995","https://openalex.org/W324549396","https://openalex.org/W328114118","https://openalex.org/W631659820","https://openalex.org/W1495442795","https://openalex.org/W1501715468","https://openalex.org/W1504369078","https://openalex.org/W1513400082","https://openalex.org/W1515235319","https://openalex.org/W1527549422","https://openalex.org/W1535750970","https://openalex.org/W1536305347","https://openalex.org/W1547889327","https://openalex.org/W1551118417","https://openalex.org/W1563146120","https://openalex.org/W1565951434","https://openalex.org/W1587685062","https://openalex.org/W1591909631","https://openalex.org/W1596848781","https://openalex.org/W1599400750","https://openalex.org/W1600305299","https://openalex.org/W1604536885","https://openalex.org/W1656279855","https://openalex.org/W1714683344","https://openalex.org/W1768641177","https://openalex.org/W1790871298","https://openalex.org/W1833855195","https://openalex.org/W1870836661","https://openalex.org/W1953755277","https://openalex.org/W1965496508","https://openalex.org/W1967855521","https://openalex.org/W1968965382","https://openalex.org/W1971393256","https://openalex.org/W1971453398","https://openalex.org/W1971475634","https://openalex.org/W1971839423","https://openalex.org/W1972547633","https://openalex.org/W1973329252","https://openalex.org/W1973466342","https://openalex.org/W1973592220","https://openalex.org/W1974751353","https://openalex.org/W1975152213","https://openalex.org/W1976029512","https://openalex.org/W1981304955","https://openalex.org/W1982321040","https://openalex.org/W1984566235","https://openalex.org/W1985486158","https://openalex.org/W1986018588","https://openalex.org/W1986490645","https://openalex.org/W1987179105","https://openalex.org/W1988972272","https://openalex.org/W1989529059","https://openalex.org/W1991047577","https://openalex.org/W1992193197","https://openalex.org/W1996303124","https://openalex.org/W1996714420","https://openalex.org/W1996723279","https://openalex.org/W1996982923","https://openalex.org/W1997513626","https://openalex.org/W1997540319","https://openalex.org/W1997863710","https://openalex.org/W1998351143","https://openalex.org/W1999347013","https://openalex.org/W1999960925","https://openalex.org/W2000914477","https://openalex.org/W2001265073","https://openalex.org/W2002353971","https://openalex.org/W2003494859","https://openalex.org/W2004118825","https://openalex.org/W2004454083","https://openalex.org/W2005573073","https://openalex.org/W2007436833","https://openalex.org/W2010346856","https://openalex.org/W2010924682","https://openalex.org/W2011194225","https://openalex.org/W2011562442","https://openalex.org/W2012769015","https://openalex.org/W2013294070","https://openalex.org/W2014054174","https://openalex.org/W2014925424","https://openalex.org/W2016981148","https://openalex.org/W2017691783","https://openalex.org/W2018503761","https://openalex.org/W2019229623","https://openalex.org/W2019282792","https://openalex.org/W2019400067","https://openalex.org/W2019872023","https://openalex.org/W2020363269","https://openalex.org/W2020555421","https://openalex.org/W2020799553","https://openalex.org/W2020869144","https://openalex.org/W2023070199","https://openalex.org/W2023361302","https://openalex.org/W2023644648","https://openalex.org/W2024706459","https://openalex.org/W2024713471","https://openalex.org/W2025505007","https://openalex.org/W2026291028","https://openalex.org/W2027313575","https://openalex.org/W2027786999","https://openalex.org/W2028804035","https://openalex.org/W2028876187","https://openalex.org/W2031699283","https://openalex.org/W2032558977","https://openalex.org/W2032566862","https://openalex.org/W2033735656","https://openalex.org/W2033774649","https://openalex.org/W2034435214","https://openalex.org/W2036391785","https://openalex.org/W2036580971","https://openalex.org/W2036625365","https://openalex.org/W2037388755","https://openalex.org/W2038435919","https://openalex.org/W2038749613","https://openalex.org/W2039561552","https://openalex.org/W2040031153","https://openalex.org/W2041413115","https://openalex.org/W2041495545","https://openalex.org/W2041614019","https://openalex.org/W2042216712","https://openalex.org/W2043808419","https://openalex.org/W2044268929","https://openalex.org/W2044846165","https://openalex.org/W2046371846","https://openalex.org/W2049265329","https://openalex.org/W2051985758","https://openalex.org/W2053114862","https://openalex.org/W2056664397","https://openalex.org/W2058152930","https://openalex.org/W2059459219","https://openalex.org/W2060542611","https://openalex.org/W2065390851","https://openalex.org/W2065818116","https://openalex.org/W2066404822","https://openalex.org/W2066743380","https://openalex.org/W2067442819","https://openalex.org/W2073292880","https://openalex.org/W2077451485","https://openalex.org/W2077473292","https://openalex.org/W2077770882","https://openalex.org/W2078341087","https://openalex.org/W2080137344","https://openalex.org/W2080762011","https://openalex.org/W2081406767","https://openalex.org/W2082438237","https://openalex.org/W2082539761","https://openalex.org/W2083397383","https://openalex.org/W2085609942","https://openalex.org/W2085866164","https://openalex.org/W2086812941","https://openalex.org/W2087035647","https://openalex.org/W2087652779","https://openalex.org/W2091894707","https://openalex.org/W2091989374","https://openalex.org/W2094253815","https://openalex.org/W2097265582","https://openalex.org/W2100805638","https://openalex.org/W2104170867","https://openalex.org/W2104571730","https://openalex.org/W2107857612","https://openalex.org/W2111984388","https://openalex.org/W2112630973","https://openalex.org/W2117960203","https://openalex.org/W2121542842","https://openalex.org/W2121798267","https://openalex.org/W2123528875","https://openalex.org/W2123885962","https://openalex.org/W2124763824","https://openalex.org/W2125026005","https://openalex.org/W2125334223","https://openalex.org/W2127633442","https://openalex.org/W2130130974","https://openalex.org/W2132295452","https://openalex.org/W2135678986","https://openalex.org/W2138419609","https://openalex.org/W2142342476","https://openalex.org/W2142686995","https://openalex.org/W2144625075","https://openalex.org/W2151734986","https://openalex.org/W2153916767","https://openalex.org/W2154126458","https://openalex.org/W2154875810","https://openalex.org/W2158115535","https://openalex.org/W2160225986","https://openalex.org/W2161907161","https://openalex.org/W2162668256","https://openalex.org/W2163069454","https://openalex.org/W2165553459","https://openalex.org/W2166010633","https://openalex.org/W2167711135","https://openalex.org/W2167948291","https://openalex.org/W2169578753","https://openalex.org/W2170912376","https://openalex.org/W2214922100","https://openalex.org/W2225388783","https://openalex.org/W2236528604","https://openalex.org/W2239608913","https://openalex.org/W2277282818","https://openalex.org/W2297358799","https://openalex.org/W2313144680","https://openalex.org/W2401231048","https://openalex.org/W2405767181","https://openalex.org/W2406760034","https://openalex.org/W2416790528","https://openalex.org/W2477934250","https://openalex.org/W2496417524","https://openalex.org/W2497421812","https://openalex.org/W2507017128","https://openalex.org/W2615749116","https://openalex.org/W2743260950","https://openalex.org/W2792659630","https://openalex.org/W3025805620","https://openalex.org/W4250789218","https://openalex.org/W4290265460"],"related_works":["https://openalex.org/W7099278495","https://openalex.org/W2100193207","https://openalex.org/W2136196895","https://openalex.org/W1164564349","https://openalex.org/W2067442819","https://openalex.org/W2154875810","https://openalex.org/W2938322338","https://openalex.org/W4399772239","https://openalex.org/W184040730","https://openalex.org/W2739865720"],"abstract_inverted_index":{"5-Aminolevulinate":[0],"synthase":[1],"(ALAS;":[2],"EC":[3],"2.3.1.37)":[4],"is":[5,126,196,216,335],"a":[6,47,74,118,127,181,292,354,389],"pyridoxal":[7,409,414,417],"5'-phosphate":[8,465],"(PLP)-dependent":[9],"enzyme":[10],"that":[11,39,100,330,422,443,497,504,518],"catalyzes":[12],"the":[13,22,77,89,131,152,163,205,331,368,371,472,507,529,535],"first":[14],"committed":[15],"step":[16],"of":[17,24,43,50,57,85,93,121,124,137,144,165,315,333,370,408,428,474,490,509,531,538],"heme":[18],"biosynthesis":[19],"in":[20,76,88,104,107,110,130,162,193,228,239,269,287,298,321,423,449,471,502,540],"animals,":[21],"condensation":[23],"glycine":[25],"and":[26,32,52,73,139,146,156,173,190,212,221,233,236,241,248,254,265,271,283,317,323,346,357,391,411,437,463,533,545],"succinyl-CoA":[27,257],"yielding":[28],"5-aminolevuliante":[29],"(ALA),":[30],"CoA,":[31],"CO2.":[33],"Murine":[34],"erythroid-specific":[35],"ALAS":[36],"(mALAS2)":[37],"variants":[38,98,150,224],"cause":[40,385],"high":[41],"levels":[42,187],"PPIX":[44,125,189],"accumulation":[45,84,268,286,448],"provide":[46,526],"new":[48],"means":[49],"targeted,":[51],"potentially":[53,546],"enhanced,":[54],"photosensitization.":[55],"Transfection":[56],"HeLa":[58,94,240,270,450],"cells":[59,95,451],"with":[60,69,176,413,478,486,506,511,543],"expression":[61,102],"plasmids":[62],"for":[63,67,256,337],"mALAS2":[64,97,101,149],"variants,":[65,246,305],"specifically":[66],"those":[68],"mutated":[70],"mitochondrial":[71],"presequences":[72],"mutation":[75],"active":[78,148],"site":[79],"loop,":[80],"caused":[81,266,284],"significant":[82],"cellular":[83,201,468],"PPIX,":[86],"particularly":[87],"membrane.":[90],"Light":[91],"treatment":[92,116,366],"expressing":[96,452],"revealed":[99,496],"results":[103,328],"an":[105,279,487],"increase":[106,170],"cell":[108],"death":[109],"comparison":[111,299],"to":[112,154,199,230,300,380,396,403,415,425],"aminolevulinic":[113],"acid":[114,374],"(ALA)":[115],"producing":[117],"similar":[119],"amount":[120],"PPIX.":[122],"Generation":[123],"crucial":[128],"component":[129],"widely":[132],"used":[133,379],"photodynamic":[134],"therapies":[135],"(PDT)":[136],"cancer":[138],"other":[140,547],"dysplasias.":[141],"The":[142,303],"delivery":[143],"stable":[145],"highly":[147],"has":[151,348,392],"potential":[153,365],"expand":[155],"improve":[157],"upon":[158,313],"current":[159],"PDT":[160],"regimes.\\nMutations":[161],"C-terminus":[164,332],"human":[166,455],"ALAS2":[167],"(hALAS2)":[168],"can":[169,384,520],"hALAS2":[171,202,214,334,404,456,469,480,503,510,519],"activity":[172,215,345,470],"are":[174,360],"associated":[175],"X-linked":[177],"erythropoietic":[178],"protoporphyria":[179],"(XLEPP),":[180],"disease":[182],"phenotypically":[183],"characterized":[184],"by":[185,399,524],"elevated":[186],"or":[188,457,483],"zinc":[191],"protoporphyrin":[192,446],"erythroblasts.":[194],"This":[195],"apparently":[197],"due":[198],"enhanced":[200],"activity,":[203],"but":[204,290],"biochemical":[206],"relationship":[207],"between":[208],"these":[209],"C-terminal":[210],"mutations":[211],"increased":[213,262,280],"not":[217],"well":[218],"understood.":[219],"HALAS2":[220],"three":[222],"XLEPP":[223,245,275,304,458,544],"were":[225,309],"studied":[226],"both":[227],"vitro":[229],"compare":[231],"kinetic":[232,344],"structural":[234,340,500],"parameters":[235],"ex":[237],"vivo":[238],"K562":[242,272],"cells.":[243,273],"Two":[244],"delAGTG,":[247],"Q548X,":[249],"exhibited":[250,291],"higher":[251],"catalytic":[252,294],"rates":[253],"affinity":[255],"than":[258],"wild-type":[259,301,307,454],"hALAS2,":[260,308,429],"had":[261,278],"transition":[263,281],"temperatures,":[264],"porphyrin":[267,285],"Another":[274],"mutation,":[276],"delAT,":[277],"temperature":[282],"mammalian":[288],"cells,":[289],"reduced":[293],"rate":[295],"at":[296],"37[deg]C":[297],"hALAS2.":[302,434],"unlike":[306],"more":[310],"structurally":[311],"responsive":[312],"binding":[314],"succinyl-CoA,":[316],"adopted":[318],"distinct":[319],"features":[320],"tertiary":[322],"PLP":[324,401,462,513],"cofactor-binding":[325],"site.":[326],"These":[327,515],"imply":[329],"important":[336],"regulating":[338],"its":[339,512],"integrity,":[341],"which":[342],"affects":[343],"stability.\\nXLEPP":[347],"only":[349],"recently":[350],"been":[351,394],"identified":[352],"as":[353,388],"blood":[355],"disorder,":[356],"thus":[358],"there":[359],"no":[361],"specific":[362],"treatments.":[363],"One":[364],"involves":[367],"use":[369,537],"antibiotic":[372],"isonicotinic":[373],"hydrazide":[375],"(isoniazid,":[376],"INH),":[377],"commonly":[378],"treat":[381],"tuberculosis.":[382],"INH":[383,430,444,498,539],"sideroblastic":[386],"anemia":[387],"side-effect":[390],"traditionally":[393],"thought":[395],"do":[397],"so":[398],"limiting":[400],"availability":[402],"via":[405],"direct":[406],"inhibition":[407,427,485],"kinase,":[410],"reacting":[412],"form":[416],"isonicotinoyl":[418],"hydrazone.":[419],"We":[420],"postulated":[421],"addition":[424],"PLP-dependent":[426],"directly":[431,522],"acts":[432],"on":[433],"Using":[435],"FACS":[436],"confocal":[438],"microscopy,":[439],"we":[440],"show":[441],"here":[442],"reduces":[445],"IX":[447],"either":[453],"variants.":[459],"In":[460],"addition,":[461],"pyridoxamine":[464],"(PMP)":[466],"restored":[467],"presence":[473],"INH.":[475],"Kinetic":[476],"analyses":[477],"purified":[479],"demonstrated":[481],"non-competitive":[482],"uncompetitive":[484],"apparent":[488],"Ki":[489],"1.5":[491],"uM.":[492],"Circular":[493],"dichroism":[494],"studies":[495,516],"triggers":[499],"changes":[501],"interfere":[505],"association":[508],"cofactor.":[514],"demonstrate":[517],"be":[521],"inhibited":[523],"INH,":[525],"insight":[527],"into":[528],"mechanism":[530],"inhibition,":[532],"support":[534],"prospective":[536],"treating":[541],"patients":[542],"cutaneous":[548],"porphyrias.":[549]},"counts_by_year":[],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
