نتایج جستجو برای: type 1 tyrosinemia

تعداد نتایج: 3647237  

Journal: :The Journal of Nutrition 2007

Journal: :Clinical chemistry 2006
Johannes Sander Nils Janzen Michael Peter Stefanie Sander Ulrike Steuerwald Ute Holtkamp Bernd Schwahn Ertan Mayatepek Friedrich K Trefz Anibh M Das

BACKGROUND False-positive and false-negative results occur in current newborn-screening programs for hepatorenal tyrosinemia, which measure tyrosine concentrations in blood spots, sometimes in combination with other metabolites, including succinylacetone. We present our experience with a newly described method for succinylacetone quantification in routine newborn screening. METHODS Succinylac...

2013
Corinne de Laet Carlo Dionisi-Vici James V Leonard Patrick McKiernan Grant Mitchell Lidia Monti Hélène Ogier de Baulny Guillem Pintos-Morell Ute Spiekerkötter

The management of tyrosinaemia type 1 (HT1, fumarylacetoacetase deficiency) has been revolutionised by the introduction of nitisinone but dietary treatment remains essential and the management is not easy. In this review detailed recommendations for the management are made based on expert opinion, published case reports and investigational studies as the evidence base is limited and there are n...

Journal: :PLoS Genetics 2006
Darius Balciunas Kirk J Wangensteen Andrew Wilber Jason Bell Aron Geurts Sridhar Sivasubbu Xin Wang Perry B Hackett David A Largaespada R. Scott McIvor Stephen C Ekker

Viruses and transposons are efficient tools for permanently delivering foreign DNA into vertebrate genomes but exhibit diminished activity when cargo exceeds 8 kilobases (kb). This size restriction limits their molecular genetic and biotechnological utility, such as numerous therapeutically relevant genes that exceed 8 kb in size. Furthermore, a greater payload capacity vector would accommodate...

Journal: :The Journal of clinical investigation 2011
Prashiela Manga Seth J Orlow

Oculocutaneous albinism (OCA) is a group of genetic disorders characterized by hypopigmentation of the skin, hair, and eyes. Affected individuals experience reduced visual acuity and substantially increased skin cancer risk. There are four major types of OCA (OCA1-OCA4) that result from disruption in production of melanin from tyrosine. Current treatment options for individuals with OCA are lim...

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