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Neutral aminoaciduria in cystathionine β-synthase-deficient mice; an animal model of homocystinuria.

Akahoshi N, Kamata S, Kubota M, Hishiki T, Nagahata Y, Matsuura T, Yamazaki C, Yoshida Y, Yamada H, Ishizaki Y, Suematsu M, Kasahara T, Ishii I (2014) Neutral aminoaciduria in cystathionine β-synthase-deficient mice; an animal model of homocystinuria. Am J Physiol Renal Physiol 306:F1462-1476. doi: 10.1152/ajprenal.00623.2013 PMID: 24761004

Summary: The authors utilized a mouse model for homocystinuria in order to examine renal amino acid reabsorbtion. Some of the immunohistochemistry experiments used anti-Met (Cat. #AB-T036). It was found that loss of cystathionine β-synthase causes hyperexcretion of both glucogenic and ketogenic neutral amino acids, as well as histidine.

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