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Elevated circulating branched chain amino acid concentration

MedGen UID:
1841537
•Concept ID:
C5826347
•
Finding
Synonym: Elevated plasma branched chain amino acids
 
HPO: HP:0008344

Definition

Concentration of a branched chain amino acid in the blood above the upper limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated circulating branched chain amino acid concentration

Conditions with this feature

Maple syrup urine disease type 1A
MedGen UID:
383668
•Concept ID:
C1855369
•
Disease or Syndrome
A maple syrup urine disease caused by mutations in BCKDHA.
See: Condition Record
Maple syrup urine disease type 1A

Recent clinical studies

Etiology

Serum branched chain amino acids: an effective indicator of diabetic kidney disease.
Liu M, Yang Y, Liu Y, Peng X, Hou Y, Zhang X, Sun H, Shan C
Front Endocrinol (Lausanne) 2023;14:1269633. Epub 2023 Nov 27 doi: 10.3389/fendo.2023.1269633. PMID: 38089615Free PMC Article
The Association between Circulating Branched Chain Amino Acids and the Temporal Risk of Developing Type 2 Diabetes Mellitus: A Systematic Review & Meta-Analysis.
Ramzan I, Ardavani A, Vanweert F, Mellett A, Atherton PJ, Idris I
Nutrients 2022 Oct 20;14(20) doi: 10.3390/nu14204411. PMID: 36297095Free PMC Article
Association of Modifiable Lifestyle Factors with Plasma Branched-Chain Amino Acid Metabolites in Women.
Hamaya R, Mora S, Lawler PR, Cook NR, Buring JE, Lee IM, Manson JE, Tobias DK
J Nutr 2022 Jun 9;152(6):1515-1524. doi: 10.1093/jn/nxac056. PMID: 35259270Free PMC Article
Inhibition of the renin-angiotensin system reduces the rise in serum aldosterone in acute coronary syndrome patients with preserved left ventricular function: observations from the AVANT GARDE-TIMI 43 trial.
Udell JA, Morrow DA, Braunwald E, Swedberg K, Bode C, Rifai N, Brunel PC, Prescott MF, Ren F, Hoffman EB, Scirica BM
Clin Chem 2013 Jun;59(6):959-67. Epub 2013 Mar 18 doi: 10.1373/clinchem.2012.199729. PMID: 23509108
Electrochemical characterisation of a conductive polymer molecularly imprinted with an Amadori compound.
Chuang SW, Rick J, Chou TC
Biosens Bioelectron 2009 Jun 15;24(10):3170-3. Epub 2009 Mar 11 doi: 10.1016/j.bios.2009.02.033. PMID: 19349156

Diagnosis

Serum branched chain amino acids: an effective indicator of diabetic kidney disease.
Liu M, Yang Y, Liu Y, Peng X, Hou Y, Zhang X, Sun H, Shan C
Front Endocrinol (Lausanne) 2023;14:1269633. Epub 2023 Nov 27 doi: 10.3389/fendo.2023.1269633. PMID: 38089615Free PMC Article
Elevated circulating level of β-aminoisobutyric acid (BAIBA) in heart failure patients with type 2 diabetes receiving sodium-glucose cotransporter 2 inhibitors.
Katano S, Yano T, Kouzu H, Nagaoka R, Numazawa R, Yamano K, Fujisawa Y, Ohori K, Nagano N, Fujito T, Nishikawa R, Ohwada W, Katayose M, Sato T, Kuno A, Furuhashi M
Cardiovasc Diabetol 2022 Dec 20;21(1):285. doi: 10.1186/s12933-022-01727-x. PMID: 36539818Free PMC Article
The Association between Circulating Branched Chain Amino Acids and the Temporal Risk of Developing Type 2 Diabetes Mellitus: A Systematic Review & Meta-Analysis.
Ramzan I, Ardavani A, Vanweert F, Mellett A, Atherton PJ, Idris I
Nutrients 2022 Oct 20;14(20) doi: 10.3390/nu14204411. PMID: 36297095Free PMC Article
Association of circulating branched-chain amino acids with risk of cardiovascular disease: A systematic review and meta-analysis.
Wang Y, Huang K, Liu F, Lu X, Huang J, Gu D
Atherosclerosis 2022 Jun;350:90-96. Epub 2022 Apr 25 doi: 10.1016/j.atherosclerosis.2022.04.026. PMID: 35576716
Branched-chain Amino Acids and Relationship With Inflammation in Youth With Obesity: A Randomized Controlled Intervention Study.
Cosentino RG, Churilla JR, Josephson S, Molle-Rios Z, Hossain MJ, Prado WL, Balagopal PB
J Clin Endocrinol Metab 2021 Oct 21;106(11):3129-3139. doi: 10.1210/clinem/dgab538. PMID: 34286837

Therapy

Elevated circulating level of β-aminoisobutyric acid (BAIBA) in heart failure patients with type 2 diabetes receiving sodium-glucose cotransporter 2 inhibitors.
Katano S, Yano T, Kouzu H, Nagaoka R, Numazawa R, Yamano K, Fujisawa Y, Ohori K, Nagano N, Fujito T, Nishikawa R, Ohwada W, Katayose M, Sato T, Kuno A, Furuhashi M
Cardiovasc Diabetol 2022 Dec 20;21(1):285. doi: 10.1186/s12933-022-01727-x. PMID: 36539818Free PMC Article
Association of circulating branched-chain amino acids with risk of cardiovascular disease: A systematic review and meta-analysis.
Wang Y, Huang K, Liu F, Lu X, Huang J, Gu D
Atherosclerosis 2022 Jun;350:90-96. Epub 2022 Apr 25 doi: 10.1016/j.atherosclerosis.2022.04.026. PMID: 35576716
Association of Modifiable Lifestyle Factors with Plasma Branched-Chain Amino Acid Metabolites in Women.
Hamaya R, Mora S, Lawler PR, Cook NR, Buring JE, Lee IM, Manson JE, Tobias DK
J Nutr 2022 Jun 9;152(6):1515-1524. doi: 10.1093/jn/nxac056. PMID: 35259270Free PMC Article
Branched-chain Amino Acids and Relationship With Inflammation in Youth With Obesity: A Randomized Controlled Intervention Study.
Cosentino RG, Churilla JR, Josephson S, Molle-Rios Z, Hossain MJ, Prado WL, Balagopal PB
J Clin Endocrinol Metab 2021 Oct 21;106(11):3129-3139. doi: 10.1210/clinem/dgab538. PMID: 34286837
The Role of Circulating Amino Acids in the Hypothalamic Regulation of Liver Glucose Metabolism.
Arrieta-Cruz I, Gutiérrez-Juárez R
Adv Nutr 2016 Jul;7(4):790S-7S. Epub 2016 Jul 15 doi: 10.3945/an.115.011171. PMID: 27422516Free PMC Article

Prognosis

Serum branched chain amino acids: an effective indicator of diabetic kidney disease.
Liu M, Yang Y, Liu Y, Peng X, Hou Y, Zhang X, Sun H, Shan C
Front Endocrinol (Lausanne) 2023;14:1269633. Epub 2023 Nov 27 doi: 10.3389/fendo.2023.1269633. PMID: 38089615Free PMC Article
Circulating Metabolites and Cardiovascular Disease in Asians with Chronic Kidney Disease.
Cai J, Chong CCY, Cheng CY, Lim CC, Sabanayagam C
Cardiorenal Med 2023;13(1):301-309. Epub 2023 Sep 5 doi: 10.1159/000533741. PMID: 37669626Free PMC Article
Divergent Survival Outcomes Associated with Elevated Branched-Chain Amino Acid Levels among Older Adults with or without Hypertension and Diabetes: A Validated, Prospective, Longitudinal Follow-Up Study.
Fung E, Ng KH, Kwok T, Lui LT, Palaniswamy S, Chan Q, Lim LL, Wiklund P, Xie S, Turner C, Elshorbagy AK, Refsum H, Leung JCS, Kong APS, Chan JCN, Järvelin MR, Woo J
Biomolecules 2023 Aug 16;13(8) doi: 10.3390/biom13081252. PMID: 37627317Free PMC Article
Longitudinal Branched-Chain Amino Acids, Lifestyle Intervention, and Type 2 Diabetes in the Finnish Diabetes Prevention Study.
Kivelä J, Meinilä J, Uusitupa M, Tuomilehto J, Lindström J
J Clin Endocrinol Metab 2022 Sep 28;107(10):2844-2853. doi: 10.1210/clinem/dgac463. PMID: 35917829Free PMC Article
A novel albumin gene mutation (R222I) in familial dysalbuminemic hyperthyroxinemia.
Schoenmakers N, Moran C, Campi I, Agostini M, Bacon O, Rajanayagam O, Schwabe J, Bradbury S, Barrett T, Geoghegan F, Druce M, Beck-Peccoz P, O'Toole A, Clark P, Bignell M, Lyons G, Halsall D, Gurnell M, Chatterjee K
J Clin Endocrinol Metab 2014 Jul;99(7):E1381-6. Epub 2014 Mar 19 doi: 10.1210/jc.2013-4077. PMID: 24646103Free PMC Article

Clinical prediction guides

Serum branched chain amino acids: an effective indicator of diabetic kidney disease.
Liu M, Yang Y, Liu Y, Peng X, Hou Y, Zhang X, Sun H, Shan C
Front Endocrinol (Lausanne) 2023;14:1269633. Epub 2023 Nov 27 doi: 10.3389/fendo.2023.1269633. PMID: 38089615Free PMC Article
Association of Modifiable Lifestyle Factors with Plasma Branched-Chain Amino Acid Metabolites in Women.
Hamaya R, Mora S, Lawler PR, Cook NR, Buring JE, Lee IM, Manson JE, Tobias DK
J Nutr 2022 Jun 9;152(6):1515-1524. doi: 10.1093/jn/nxac056. PMID: 35259270Free PMC Article
A novel albumin gene mutation (R222I) in familial dysalbuminemic hyperthyroxinemia.
Schoenmakers N, Moran C, Campi I, Agostini M, Bacon O, Rajanayagam O, Schwabe J, Bradbury S, Barrett T, Geoghegan F, Druce M, Beck-Peccoz P, O'Toole A, Clark P, Bignell M, Lyons G, Halsall D, Gurnell M, Chatterjee K
J Clin Endocrinol Metab 2014 Jul;99(7):E1381-6. Epub 2014 Mar 19 doi: 10.1210/jc.2013-4077. PMID: 24646103Free PMC Article
Inhibition of the renin-angiotensin system reduces the rise in serum aldosterone in acute coronary syndrome patients with preserved left ventricular function: observations from the AVANT GARDE-TIMI 43 trial.
Udell JA, Morrow DA, Braunwald E, Swedberg K, Bode C, Rifai N, Brunel PC, Prescott MF, Ren F, Hoffman EB, Scirica BM
Clin Chem 2013 Jun;59(6):959-67. Epub 2013 Mar 18 doi: 10.1373/clinchem.2012.199729. PMID: 23509108
Insulin resistance and the metabolism of branched-chain amino acids in humans.
Adeva MM, Calviño J, Souto G, Donapetry C
Amino Acids 2012 Jul;43(1):171-81. Epub 2011 Oct 8 doi: 10.1007/s00726-011-1088-7. PMID: 21984377

Recent systematic reviews

The Association between Circulating Branched Chain Amino Acids and the Temporal Risk of Developing Type 2 Diabetes Mellitus: A Systematic Review & Meta-Analysis.
Ramzan I, Ardavani A, Vanweert F, Mellett A, Atherton PJ, Idris I
Nutrients 2022 Oct 20;14(20) doi: 10.3390/nu14204411. PMID: 36297095Free PMC Article
Association of circulating branched-chain amino acids with risk of cardiovascular disease: A systematic review and meta-analysis.
Wang Y, Huang K, Liu F, Lu X, Huang J, Gu D
Atherosclerosis 2022 Jun;350:90-96. Epub 2022 Apr 25 doi: 10.1016/j.atherosclerosis.2022.04.026. PMID: 35576716

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