Warning: Undefined array key "mm" in /www/wwwroot/www.ai-bt.com/si.php on line 10 Deprecated: trim(): Passing null to parameter #1 ($string) of type string is deprecated in /www/wwwroot/www.ai-bt.com/si.php on line 10 HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN.

Literature DB >> 14163785

HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN.

Abstract

Cystathionine was absent in the cerebrum of a patient with homocystinuria. This supports the theory that the enzymatic defect in homocystinuria is deficiency of cystathionine synthetase.

Entities: Chemical Disease Species

Keywords: AMINO ACIDS; AMINOACIDURIA, RENAL; BRAIN; CHILD; CHROMATOGRAPHY; CYSTINE; HYDRO-LYASES; LIVER ENZYMOLOGY; METABOLIC DISEASES; METHIONINE; NERVE TISSUE PROTEINS; SERINE; TAURINE

Mesh：

Substances：

Year: 1964 PMID： 14163785 DOI： 10.1126/science.145.3632.588

Source DB: PubMed Journal: Science ISSN： 0036-8075 Impact factor: 47.728

Keyword Cloud
Cited

10 in total

1. Homocystinuria: a study with low-methionine diet in three patients.

Authors: I B Sardharwalla; S H Jackson; H D Hawke; A Sass-Kortsak
Journal: Can Med Assoc J Date: 1968-10-19 Impact factor: 8.262

2. Homocystinuria.

Authors: D C Cusworth; C E Dent
Journal: Biochem J Date: 1969-02 Impact factor: 3.857

3. Homocystinuria and the passing of the one gene-one enzyme concept of disease.

Authors: G L Spaeth; G W Barber
Journal: Trans Am Ophthalmol Soc Date: 1978

4. Homocystine-induced arteriosclerosis. The role of endothelial cell injury and platelet response in its genesis.

Authors: L A Harker; R Ross; S J Slichter; C R Scott
Journal: J Clin Invest Date: 1976-09 Impact factor: 14.808

5. The effects of chronic isoniazid intoxication on motor end plate sprouting in rat sternocostalis muscle and on responses to partial denervation and local botulinum toxin.

Authors: S Kemplay; J B Cavanagh
Journal: Acta Neuropathol Date: 1984 Impact factor: 17.088

6. Postnatal Subacute Benzo(a)Pyrene Exposure Caused Neurobehavioral Impairment and Metabolomic Changes of Cerebellum in the Early Adulthood Period of Sprague-Dawley Rats.

Authors: Chunlin Li; Jing Wang; Qiuping Su; Kai Yang; Chengzhi Chen; XueJun Jiang; Tingli Han; Shuqun Cheng; Tingting Mo; Ruiyuan Zhang; Bin Peng; Yuming Guo; Philip N Baker; Baijie Tu; Yinyin Xia
Journal: Neurotox Res Date: 2017-12-01 Impact factor: 3.911

HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN.

1. Homocystinuria: a study with low-methionine diet in three patients.

2. Homocystinuria.

3. Homocystinuria and the passing of the one gene-one enzyme concept of disease.

4. Homocystine-induced arteriosclerosis. The role of endothelial cell injury and platelet response in its genesis.

5. The effects of chronic isoniazid intoxication on motor end plate sprouting in rat sternocostalis muscle and on responses to partial denervation and local botulinum toxin.

6. Postnatal Subacute Benzo(a)Pyrene Exposure Caused Neurobehavioral Impairment and Metabolomic Changes of Cerebellum in the Early Adulthood Period of Sprague-Dawley Rats.

7. Homocystinuria due to cystathionine synthase deficiency: the metabolism of L-methionine.

8. The effectiveness of correcting abnormal metabolic profiles.

Review 9. Structural Basis for Allostery in PLP-dependent Enzymes.

Review 10. The chronological evolution of small organic molecular fluorescent probes for thiols.