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Experiment

PEG-CBS treatment in TG I278T mice
Summary Data Summary
Investigator Majtan, Tomas
Description transgenic I278T mice, one of the mouse models for cystathionine beta-synthase
(CBS)-deficient homocystinuria, were treated with an experimental enzyme
replacement therapy for homocystinuria, designated as PEG-CBS, and compared to
untreated affected homozygous and untreated healthy heterozygous mice.

Transgenic mice are a mouse model of homocystinuria, an inherited metabolic
disorder resulting from the lack of cystathionine beta-synthase (CBS) activity.

Applicable research area(s): Metabolism
Status Completed
Public Release 8/20/2017
Animal Age Measured In: week(s) post-natal (w)
Flags has-data-flagSame StrainAnimal CountExperiment Description
Data Analysis
TypeCount
Animals14
Experimental Conditions8
Catalog Items2
Curation Info (# flags)3
Phenotype Assays4
Phenotype Measurements56
Histology Images0
Publications0


Animals

Strain NameCommon NameFemalesMalesUnknown
Tg-I278T
4
9
0
Tg-I278TTg/+
0
1
0

Experimental Factors
 Categorical Values
Name / AbbreviationDescription

Experimental Factor: Drug Administered

 PEG-CBS
Polyethylene glycol cystathionine beta-synthase
 Vehicle
Vehicle - Control

Experimental Factor: Drug Dose Units

 mg/kg 3X per wk
milligrams per kilograms 3 times per week

Experimental Factor: Experimental Group

 Control
This animal belongs to the control group for the experiment.
 Experiment
This animal belongs to experimental group that is homozygous for gene manipulations.
 Experiment-Het
This animal belongs to the experimental group and is heterozygous wild type/mutation for the affected locus.

Experimental Factor: Feeding Group

 Fed
Animals are allowed to have food.

Experimental Factor: Mouse Diet

 Teklad - 2918
Harlan-Teklad 2918 (Irradiated) 18.6% protein (Calories : 24% protein, 18% Fat, 58% Carbohydrate) Ingredients—Ground wheat, ground corn, wheat middlings, dehulled soybean meal, corn gluten meal, soybean oil, calcium carbonate, brewers dried yeast,...

Experimental Factor: Tissue Source

 plasma (lithium heparin)
plasma (lithium heparin)



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