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GM20161 male affected Glucosidase acid alpha sample

Identifiers
BioSample: SAMN00805692; Coriell: GM20161
Organism
Homo sapiens (human)
cellular organisms; Eukaryota; Opisthokonta; Metazoa; Eumetazoa; Bilateria; Deuterostomia; Chordata; Craniata; Vertebrata; Gnathostomata; Teleostomi; Euteleostomi; Sarcopterygii; Dipnotetrapodomorpha; Tetrapoda; Amniota; Mammalia; Theria; Eutheria; Boreoeutheria; Euarchontoglires; Primates; Haplorrhini; Simiiformes; Catarrhini; Hominoidea; Hominidae; Homininae; Homo
Attributes
cell lineGM20161
sexMale
cell typeFibroblast
raceCaucasian
ethnicityPORTUGUESE
age2 MO
GeneGAA
affected_byGlucosidase acid alpha
MutationCYS647TRP
collectionNIGMS Human Genetic Cell Repository
Tags
GAA
Glucosidase acid alpha
Glycogen storage disease ii
Description

Clinically affected;cardiomegaly;macroglossia;hepatomegaly;hypotonia;died before one year of age;alpha glucosidase activity = 2.9 ug/mg prot/hr (normal = 137.1 to 1025.9);donor subject is homozygous for a C>G transversion at nucleotide 1941 in exon 14 of the GAA gene 1941C>G resulting in a substitution of tryptophan for cysteine at codon 647 Cys647Trp(C647W) .

Submission
Coriell; 2012-03-05
Accession:
SAMN00805692
ID:
805692

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