Maple Syrup Urine Disease Model

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Wild Mouse 750
Wild Mouse 750
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huPBMC-NCGhuHSC-NCG-XhuHSC-NCG-hIL15huHSC-NCG
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Wild Mouse 765
Wild Mouse 765

Maple syrup urine disease (MSUD) is an autosomal recessive disorder, characterized by a deficiency of the branched-chain alpha-keto acid dehydrogenase, which results in the accumulation of branched-chain amino acids leucine, isoleucine, and valine.

 

● Genetically Engineered MSUD Mouse Model

Strain No.
 Strain Name Strain Type Description
T049844 Bcat2-KO Knockout Homozygous Bcat-KO mice showed impaired survival and decreased body weight. Plasma branch chain amino acid (BCAA) levels are significantly elevated in both male and female homozygous Bcat-KO mice, which mimics the phenotype of maple syrup urine disease