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 Quantitation of the enzymically deficient cross reacting material in GM1 gangliosidoses.

Literature DB >> 412418

Quantitation of the enzymically deficient cross reacting material in GM1 gangliosidoses.

Abstract

Normal quantities of GM1 beta-galactosidase cross reacting material (CRM) (0.31-0.47 microgram/mg protein) were detected by a sensitive radial immunodiffusion assay in skin fibroblasts from patients with GM1 gangliosidosis type 1 and adult variants, whereas elevated levels were found in GM1 gangliosidosis type 2 (0.41-0.72 microgram/mg protein). The specific activity of the immunologically CRM towards GM1 ganglioside of normal fibroblasts was about 500 times that of type 1, 100 times that of type 2, and 30 times that of the adult variants.

Entities: Chemical Disease Species

Mesh：

Substances：

Year: 1977 PMID： 412418 PMCID： PMC1685510

Source DB: PubMed Journal: Am J Hum Genet ISSN： 0002-9297 Impact factor: 11.025

20 in total

1. Protein measurement with the Folin phenol reagent.

Authors: O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal: J Biol Chem Date: 1951-11 Impact factor: 5.157

2. An improved method for the identification of patients and carriers of Krabbe's disease.

Authors: D A Wenger; M Sattler; C Clark; H McKelvey
Journal: Clin Chim Acta Date: 1974-10-30 Impact factor: 3.786

3. Letter: Beta-galactosidase deficiency in young adults.

Authors: D A Wenger; S I Goodman; G G Myers
Journal: Lancet Date: 1974-11-30 Impact factor: 79.321

4. Generalized gangliosidosis.

Authors: J O'Brien
Journal: J Pediatr Date: 1969-08 Impact factor: 4.406

5. Late infantile systemic lipidosis. Major monosialogangliosidosis. Delineation of two types.

Authors: D M Derry; J S Fawcett; F Andermann; L S Wolfe
Journal: Neurology Date: 1968-04 Impact factor: 9.910

6. Immunological studies of beta galactosidase in normal human liver and in GM1 gangliosidosis.

Authors: M Meisler; M C Rattazzi
Journal: Am J Hum Genet Date: 1974-11 Impact factor: 11.025

7. Nature of the mutation in adult beta-galactosidase deficient patients.

Authors: J S O'Brien; A G Norden
Journal: Am J Hum Genet Date: 1977-03 Impact factor: 11.025

8. Hydrolysis of GM1-ganglioside by human liver beta-galactosidase isoenzymes.

Authors: M W Ho; P Cheetham; D Robinson
Journal: Biochem J Date: 1973-10 Impact factor: 3.857

9. Generalized gangliosidosis: beta-galactosidase deficiency.

Authors: S Okada; J S O'Brien
Journal: Science Date: 1968-05-31 Impact factor: 47.728

10. Localized beta-galactosidase deficiency. Occurrence in cerebellar ataxia with myoclonus epilepsy and macular cherry-red spot--a new variant of GM1-gangliosidosis?

Authors: A Yamamoto; S Adachi; S Kawamura; M Takahashi; T Kitani
Journal: Arch Intern Med Date: 1974-10

8 in total

1. Pitfalls in the use of artificial substrates for the diagnosis of Gaucher's disease.

Authors: Y Ben-Yoseph; H L Nadler
Journal: J Clin Pathol Date: 1978-11 Impact factor: 3.411

2. GM1-Gangliosidosis: a molecular abnormality of acid beta-galactosidase in fibroblasts.

Authors: T Furuya; Y Suzuki
Journal: J Inherit Metab Dis Date: 1984 Impact factor: 4.982

3. Acid lipase cross-reacting material in Wolman disease and cholesterol ester storage disease.

Authors: B K Burton; S P Reed
Journal: Am J Hum Genet Date: 1981-03 Impact factor: 11.025

4. Demonstration of altered acidic hydrolases in fibroblasts from patients with mucolipidosis II by lectin titration.

Authors: R Rousson; Y Ben-Yoseph; M B Fiddler; H L Nadler
Journal: Biochem J Date: 1979-06-15 Impact factor: 3.857

5. The interrelations between high- and low-molecular weight forms of normal and mutant (Krabbe-disease) galactocerebrosidase.

Authors: Y Ben-Yoseph; M Hungerford; H L Nadler
Journal: Biochem J Date: 1980-07-01 Impact factor: 3.857