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GM17482 female affected HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, BETA SUBUNIT sample

Identifiers
BioSample: SAMN00804900; Coriell: GM17482
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 lineGM17482
sexFemale
cell typeFibroblast
raceCaucasian
age13 YR
GeneHADHB
affected_byHYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, BETA SUBUNIT
MutationARG247HIS
MutationARG61HIS
collectionNIGMS Human Genetic Cell Repository
Tags
Trifunctional protein deficiency
HADHB
HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, BETA SUBUNIT
Description

Clinically affected;presented at 3 months of age with hypoglycemia;hyperammonemia;mild liver dysfunction and 3-hydroxydicarboxylic aciduria;diagnosed at age 13 with recurrent hypoglycemia;progressive myopathy;and peripheral neuropathy;plasma acylcarnitine profile characteristic of LCHAD deficiency and enzyme testing showed complete trifunctional protein deficiency patient #3 in Am J Hum Genet 58:979-988 (1996) - sex in this publication was incorrectly listed as male;donor subject is a compound heterozygote;one allele has a G>A transition at nucleotide 182 of the HADHB gene 182G>A resulting in a substitution of histidine for arginine at codon 61 Arg61His (R61H) and a second allele has a G>A transition at nucleotide 740 of the HADHB gene 740G>A resulting in a substitution of histidine for arginine at codon 247 Arg247His (R247H) .

Submission
Coriell; 2012-03-05
Accession:
SAMN00804900
ID:
804900

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