PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 22903887-4 2012 Here, we show that AAV2/8-mediated, ETHE1-gene transfer to the liver of a genetically, metabolically and clinically faithful EE mouse model resulted in full restoration of SDO activity, correction of plasma thiosulfate, a biomarker reflecting the accumulation of H(2)S, and spectacular clinical improvement. Thiosulfates 207-218 ethylmalonic encephalopathy 1 Mus musculus 36-41 19136963-6 2009 We found that thiosulfate was excreted in massive amounts in urine of both Ethe1(-/-) mice and humans with ethylmalonic encephalopathy. Thiosulfates 14-25 ethylmalonic encephalopathy 1 Mus musculus 75-80 19136963-7 2009 High thiosulfate and sulfide concentrations were present in Ethe1(-/-) mouse tissues. Thiosulfates 5-16 ethylmalonic encephalopathy 1 Mus musculus 60-65