Literature DB >> 819629

Excretion of guanidino-derivates in urine of hyperargininemic patients.

P Wiechert, J Mortelmans, F Lavinha, R Clara, H G Terheggen, A Lowenthal.   

Abstract

Quantitative determinations were made for seven guanidino-derivates in urine of patients affected with hyperargininemia, lysine cystinuria, healthy children and adults, goats and monkey. The amount of all seven guanidino-derivates excreted in the urine with the exception of guanidino-succinic acid was large in the case of hyperargininemic patients. Guanidino-succinic acid is found in traces in the urine of these patients, even after arginine loading tests. There is apparently no similarity concerning the guanidino-derivates between the patients with hyperargininemia, a pathological status, and the arginase deficiency in goats and monkeys, a physiological phenomenon.

Entities:  

Mesh:

Substances:

Year:  1976        PMID: 819629

Source DB:  PubMed          Journal:  J Genet Hum        ISSN: 0021-7743


  4 in total

1.  Alterations in neuronal metabolism contribute to the pathogenesis of prion disease.

Authors:  Julie-Myrtille Bourgognon; Jereme G Spiers; Hannah Scheiblich; Alexey Antonov; Sophie J Bradley; Andrew B Tobin; Joern R Steinert
Journal:  Cell Death Differ       Date:  2018-06-18       Impact factor: 15.828

2.  Lipid nanoparticle-targeted mRNA therapy as a treatment for the inherited metabolic liver disorder arginase deficiency.

Authors:  Brian Truong; Gabriella Allegri; Xiao-Bo Liu; Kristine E Burke; Xuling Zhu; Stephen D Cederbaum; Johannes Häberle; Paolo G V Martini; Gerald S Lipshutz
Journal:  Proc Natl Acad Sci U S A       Date:  2019-09-09       Impact factor: 11.205

3.  Human recombinant arginase enzyme reduces plasma arginine in mouse models of arginase deficiency.

Authors:  Lindsay C Burrage; Qin Sun; Sarah H Elsea; Ming-Ming Jiang; Sandesh C S Nagamani; Arthur E Frankel; Everett Stone; Susan E Alters; Dale E Johnson; Scott W Rowlinson; George Georgiou; Brendan H Lee
Journal:  Hum Mol Genet       Date:  2015-09-10       Impact factor: 6.150

4.  AAV-based gene therapy prevents neuropathology and results in normal cognitive development in the hyperargininemic mouse.

Authors:  E K Lee; C Hu; R Bhargava; R Ponnusamy; H Park; S Novicoff; N Rozengurt; B Marescau; P De Deyn; D Stout; L Schlichting; W W Grody; S D Cederbaum; G S Lipshutz
Journal:  Gene Ther       Date:  2013-02-07       Impact factor: 5.250

  4 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.