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The Role of Branched Chain Amino Acids in Human Disease

The Role of Branched Chain Amino Acids in Human Disease
Reported by
Kari Fischer

Posted July 20, 2020

Presented By

The Biochemical Pharmacology Discussion Group

Branched chain amino acids (BCAAs) are essential protein building blocks that are used as metabolic fuel to promote cellular growth and replication. BCAAs (leucine, isoleucine, and valine) and catabolic intermediates also serve as signaling molecules to communicate the nutritional status of the organism to the cell. Genetic loss of branched-chain a-keto acid dehydrogenase—a crucial component of BCAA catabolism—is associated with profound inborn errors of metabolism such as maple syrup urine disease and other neurological disorders. In addition, recent clinical and preclinical studies have implicated altered BCAA metabolism in multiple diseases including diabetes/metabolic syndrome, heart failure, and cancer. However, there is a lack of understanding regarding the mechanisms underlying the alterations in BCAA catabolism, how this dysfunction leads to disease, and whether aberrant BCAA metabolism is causal to or is merely a consequence of disease.

At the Branched Chain Amino Acids and Human Disease conference, held virtually on May 14, 2020, multidisciplinary scientists reviewed the fundamentals of BCAA metabolism and the contributions of BCAA dysregulation in several human diseases.

In this eBriefing, You’ll Learn:

  • What is known about BCAA metabolism in various systems
  • The existing knowledge gaps in BCAA research in human disease
  • Potential therapeutic nodes of intervention to ameliorate human diseases
Fundamentals of BCAA Metabolism
Translating BCAA Metabolism for Human Disease

The Role of Branched Chain Amino Acids in Human Disease - Part 2

David Chuang/Tracy Anthony/Zoltan Arany (University of Texas Southwestern Medical Center/Rutgers University/University of Pennsylvania)

Further Readings


Neinast MD, Jang C, Hui S, et al

Cell Metab. 2019;29(2):417-429.e4

BCAA Metabolism and Cardiovascular Diseases