نتایج جستجو برای: hyperprolinemia

تعداد نتایج: 76  

Journal: :Human molecular genetics 2002
Hélène Jacquet Grégory Raux Florence Thibaut Bernadette Hecketsweiler Emmanuelle Houy Caroline Demilly Sadeq Haouzir Gabrielle Allio Gael Fouldrin Valérie Drouin Jacqueline Bou Michel Petit Dominique Campion Thierry Frébourg

The increased prevalence of schizophrenia among patients with the 22q11 interstitial deletion associated with DiGeorge syndrome has suggested the existence of a susceptibility gene for schizophrenia within the DiGeorge syndrome chromosomal region (DGCR) on 22q11. Screening for genomic rearrangements of 23 genes within or at the boundaries of the DGCR in 63 unrelated schizophrenic patients and 6...

Journal: :Human molecular genetics 2007
Grégory Raux Emilie Bumsel Bernadette Hecketsweiler Therese van Amelsvoort Janneke Zinkstok Sylvie Manouvrier-Hanu Carole Fantini Georges-Marie M Brévière Gabriella Di Rosa Giuseppina Pustorino Annick Vogels Ann Swillen Solenn Legallic Jacqueline Bou Gaelle Opolczynski Valérie Drouin-Garraud Marie Lemarchand Nicole Philip Aude Gérard-Desplanches Michèle Carlier Anne Philippe Marie Christine Nolen Delphine Heron Pierre Sarda Didier Lacombe Cyril Coizet Yves Alembik Valérie Layet Alexandra Afenjar Didier Hannequin Caroline Demily Michel Petit Florence Thibaut Thierry Frebourg Dominique Campion

Microdeletions of the 22q11 region, responsible for the velo-cardio-facial syndrome (VCFS), are associated with an increased risk for psychosis and mental retardation. Recently, it has been shown in a hyperprolinemic mouse model that an interaction between two genes localized in the hemideleted region, proline dehydrogenase (PRODH) and catechol-o-methyl-transferase (COMT), could be involved in ...

Journal: :Journal of Inborn Errors of Metabolism and Screening 2017

Journal: :The Tohoku journal of experimental medicine 1987
K Oyanagi A Tsuchiyama Y Itakura Y Tamura T Nakao S Fujita H Shiono

A severe mentally retarded infant with type I hyperprolinemia associated with chromosomal abnormality is reported. The patient had a characteristic facial appearance of hyperprolinemia and suffered from convulsions after the age of 10 months. The child developed severe mental and motor retardation. The karyotype of the patient revealed partial duplication of the short arm in chromosome 10 using...

Journal: :Annals of clinical and laboratory science 2013
Mi-Ae Jang Byung Cheol Kim Chang-Seok Ki Soo-Youn Lee Jong-Won Kim Tae Youn Choi Dong Hwan Lee Junghan Song Yong-Wha Lee Hyung-Doo Park

BACKGROUND Hyperprolinemia is a rare inherited metabolic disorder characterized by a high proline level in blood and/or urine and various neuropsychiatric symptoms. Type I hyperprolinemia is caused by a proline oxidase deficiency, which is encoded by the PRODH gene on chromosome 22q11. Herein, we present a study of Korean patients with type I hyperprolinemia who were diagnosed during newborn sc...

2013
DAVID VALLE M. PHANG

from five obligate heterozygotes for type II hyperprolinemia. We also demonstrated a reduction in leukocyte AL-pyrroline-5-carboxylic acid dehydrogenase activity in three successive generations of a family. A portion of this work was presented at the Annual Meeting of The Society for Pediatric Research in Washiilgton, D. C., 3 May 1974, and at the National Meeting of The American Society for Cl...

Journal: :Clinical chemistry 1978
Y Yamaguchi

This procedure for determining serum proline in patients with hyperprolinemia involves protein precipitation (Folin-Wu method), color development with isatin, extraction of the color with methylene chloride and measurement of its absorbance at 600 nm. The specificity and analytical recovery show the method to be suitable for this use.

Journal: :journal of paramedical sciences 0
saeid rahman zadeh department of biology , faculty of sciences , islamic azad university, science & research campus , tehran hamid shahbaz mohammadi department of biochemistry pasteur institute of iran morteza karimipour molecular medicine group, biotechnology research centre, pasteur institute of iran, tehran, iran saeed heidari keshel proteomics research center, shahid beheshti university of medical sciences, tehran, iran

schizophrenia is a complicated, debilitative mental disorder. evidence is emerging for the association of polymorphisms in prodh gene and increased risk of schizophrenia. in the present research, we investigated relationship between of this gene and schizophrenia disease by means of a gene polymorphism using pcr-rflp technique.150 persons suffering from acute schizophrenia and 160 healthy perso...

Journal: :The Journal of clinical investigation 1964
C R SCRIVER M L EFRON I A SCHAFER

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