| lysosomal α. |
glucan 1,4-α-glucosidase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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|---|---|
| lysosomal c. |
cysteine-type carboxypeptidase (def. 2).
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| lysosomal e. |
lysosomal storage disease.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| lysosomal storage d. |
any inborn error of metabolism having four characteristics: (1) a defect in a specific lysosomal hydrolase; (2) intracellular accumulation of the unmetabolized substrate; (3) clinical progression affecting multiple tissues and organs; (4) considerable phenotypic variation within a disease. All but two of the lysosomal storage disorders are of autosomal recessive inheritance. The term comprises the mucolipidoses, mucopolysaccharidoses, disorders of glycoprotein degradation, lipase deficiencies, ceramidase deficiency (Farber's disease), α-galactosidase A deficiency (Fabry's disease), lipidoses, and gangliosidoses. Called also lysosomal enzymopathy and inborn lysosomal d. See also inborn errors of metabolism, under error.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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