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Select item 14589841.

GeneReviews® [Internet].

Adam MP, Bick S, Mirzaa GM, et al., editors.

Seattle (WA): University of Washington, Seattle; 1993-2026.

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Select item 44036682.

StatPearls [Internet].

Treasure Island (FL): StatPearls Publishing; 2026 Jan-.

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Select item 54658903.

Toxicological Profile for Phosphate Ester Flame Retardants.

Atlanta (GA): Agency for Toxic Substances and Disease Registry (US); 2012 Sep.

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Select item 52109624.

Modelling of hypothetical SARS-CoV-2 point of care tests for routine testing in residential care homes: rapid cost-effectiveness analysis.

Stevenson M, Metry A, Messenger M.

Southampton (UK): NIHR Journals Library; 2021 Jun. (Health Technology Assessment, No. 25.39.)

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Select item 56348725.

Niraparib and Abiraterone Acetate (Akeega): CADTH Reimbursement Review: Therapeutic area: Metastatic castration-resistant prostate cancer [Internet].

Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2024 Apr.

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Select item 53416166.

Safety of disinvestment in mid- to late-term follow-up post primary hip and knee replacement: the UK SAFE evidence synthesis and recommendations.

Kingsbury SR, Smith LK, Czoski Murray CJ, et al.

Southampton (UK): National Institute for Health and Care Research; 2022 Jun. (Health and Social Care Delivery Research, No. 10.16.)

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Select item 43860047.

The assessment and appraisal of regenerative medicines and cell therapy products: an exploration of methods for review, economic evaluation and appraisal.

Hettle R, Corbett M, Hinde S, et al.

Southampton (UK): NIHR Journals Library; 2017 Feb. (Health Technology Assessment, No. 21.7.)

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Select item 29911048.

Dietary Reference Intakes for Thiamin, Riboflavin, Niacin, Vitamin B6, Folate, Vitamin B12, Pantothenic Acid, Biotin, and Choline.

Institute of Medicine (US) Standing Committee on the Scientific Evaluation of Dietary Reference Intakes and its Panel on Folate, Other B Vitamins, and Choline.

Washington (DC): National Academies Press (US); 1998.

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Select item 18813829.

Basic Neurochemistry: Molecular, Cellular and Medical Aspects. 6th edition.

Siegel GJ, Agranoff BW, Albers RW, et al., editors.

Philadelphia: Lippincott-Raven; 1999.

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Select item 462959210.

Guideline: Fortification of Rice with Vitamins and Minerals as a Public Health Strategy.

Geneva: World Health Organization; 2018.

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Select item 330887211.

A Framework to Guide Selection of Chemical Alternatives.

Committee on the Design and Evaluation of Safer Chemical Substitutions: A Framework to Inform Government and Industry Decision; Board on Chemical Sciences and Technology; Board on Environmental Studies and Toxicology; Division on Earth and Life Studies; National Research Council.

Washington (DC): National Academies Press (US); 2014 Oct 29.

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Select item 518078612.

Modelling of hypothetical SARS-CoV-2 point-of-care tests on admission to hospital from A&E: rapid cost-effectiveness analysis.

Stevenson M, Metry A, Messenger M.

Southampton (UK): NIHR Journals Library; 2021 Mar. (Health Technology Assessment, No. 25.21.)

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Select item 494716013.

Screening for Cognitive Impairment in Older Adults: An Evidence Update for the U.S. Preventive Services Task Force [Internet].

Patnode CD, Perdue LA, Rossom RC, et al.

Rockville (MD): Agency for Healthcare Research and Quality (US); 2020 Feb. (Evidence Synthesis, No. 189.)

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Select item 481264614.

Clinical Review Report: Cerliponase Alfa (Brineura): (Biomarin Pharmaceutical (Canada) Inc.): Indication: For the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) disease, also known as tripeptidyl peptidase 1 (TPP1) deficiency [Internet].

Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2019 Jun.

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Select item 494989615.

Promoting Positive Adolescent Health Behaviors and Outcomes: Thriving in the 21st Century.

National Academies of Sciences, Engineering, and Medicine; Health and Medicine Division; Division of Behavioral and Social Sciences and Education; Board on Children, Youth, and Families; Committee on Applying Lessons of Optimal Adolescent Health to Improve Behavioral Outcomes for Youth; Kahn NF, Graham R, editors.

Washington (DC): National Academies Press (US); 2019 Dec 12.

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Select item 430428616.

Gene Drives on the Horizon: Advancing Science, Navigating Uncertainty, and Aligning Research with Public Values.

Committee on Gene Drive Research in Non-Human Organisms: Recommendations for Responsible Conduct; Board on Life Sciences; Division on Earth and Life Studies; National Academies of Sciences, Engineering, and Medicine.

Washington (DC): National Academies Press (US); 2016 Jul 28.

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Select item 522256417.

Nutrition and HIV: Epidemiological Evidence to Public Health.

Mehta S, Finkelstein JL, editors.

New York (NY): CRC Press; 2018 May 15.

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Select item 550183818.

Toxicological Profile for Hydraulic Fluids.

Atlanta (GA): Agency for Toxic Substances and Disease Registry (US); 1997 Sep.

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Select item 186900819.

Sequence - Evolution - Function: Computational Approaches in Comparative Genomics.

Koonin EV, Galperin MY.

Boston: Kluwer Academic; 2003.

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Select item 141528020.

Madame Curie Bioscience Database [Internet].

Austin (TX): Landes Bioscience; 2000-2013.

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Supplemental Content

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Fig. 1.26

Schematic diagram of a protein active site with a coenzyme (thiamine pyrophosphate) as the catalytic element of pyruvate decarboxylase

The Structure and Function of Living Organisms.

Konieczny L, Roterman-Konieczna I, Spólnik P. Systems Biology: Functional Strategies of Living Organisms [Internet]. 2nd edition. Cham (CH): Springer; 2023.
Figure 3-58Coenzymes

Coenzymes, such as thiamine pyrophosphate (TPP), shown here in gray, are small molecules that bind to an enzyme's surface and enable it to catalyze specific reactions. The reactivity of TPP depends on its "acidic" carbon atom, which readily exchanges its hydrogen atom for a carbon atom of a substrate molecule. Other regions of the TPP molecule act as "handles" by which the enzyme holds the coenzyme in the correct position. Coenzymes presumably evolved first in an "RNA world," where they were bound to RNA molecules to help with catalysis (discussed in Chapter 1).

Proteins as Catalysts .

Alberts B, Bray D, Lewis J, et al. Molecular Biology of the Cell. 3rd edition. New York: Garland Science; 1994.
Figure 3.

Pyruvate metabolism. Circled compounds represent substrates for pyruvate synthesis. Major end-products are boxed. E. faecalis genes that code enzymes for reactions, cofactors, and gradients are shown: pyk, pyruvate kinase; mez malic enzyme; cit, citrate lyase; ACP, acyl carrier protein (γ-subunit), oad, oxaloacetate decarboxylase; ΔµNA⁺, transmembrane sodium gradient; por, pyruvate decarboxylase; fd_red, reduced ferredoxin; fd_ox, oxidized ferredoxin; pdh pyruvate dehydrogenase complex including E1, E2 and E3 subunits; TTP, thiamine pyrophosphate; ldh L-(+)-lactate dehydrogenase; alsS, α-acetolactate synthase; alsD, α-acetolactate decarboxylase; pfl, pyruvate formate-lyase; adhE, aldehyde-alcohol dehydrogenase; ack, acetokinase; pta, phosphoacetyltransferase. Exact stoichiometry is not indicated and reaction details are omitted for clarity.

The Physiology and Metabolism of Enterococci.

Gilmore MS, Clewell DB, Ike Y, et al., editors. Enterococci: From Commensals to Leading Causes of Drug Resistant Infection [Internet]. Boston: Massachusetts Eye and Ear Infirmary; 2014-.
Figure 44-1

Major pathways of branched-chain amino acid metabolism. Maple syrup urine disease is caused by a congenital deficiency of reaction 2. Many of the primary organic acidurias, for example, isovaleric acidemia and methylmalonic acidemia, are referable to inherited defects of enzymes involved in the oxidation of organic acids derived from the branched-chain amino acids. Enzymes: 1, branched-chain amino acid transaminase; 2, branched-chain amino acid decarboxylase; 3, isovaleryl-CoA dehydrogenase; 4, glycine-N-acylase; 5, 3-methylcrotonyl-CoA carboxylase; 6, crotonase; 7, 3-methylglutaconyl-CoA hydratase; 8, 3-OH-3-methylglutaryl-CoA lyase; 9, 2-ketothiolase; 10, isobutyryl-CoA dehydrogenase; 11, propionyl-CoA carboxylase; 12, methylmalonyl-CoA mutase; 13, 3-OH-isobutyryl-CoA deacylase. TPP, thiamine pyrophosphate; LipA, lipoic acid; ETF, electron transfer flavoprotein; AdoB₁₂, adenosylcobalamin; IVA, isovaleric acid; IVG, isovalerylglycine; TCA, tricarboxylic acid.

Diseases of Amino Acid Metabolism.

Siegel GJ, Agranoff BW, Albers RW, et al., editors. Basic Neurochemistry: Molecular, Cellular and Medical Aspects. 6th edition. Philadelphia: Lippincott-Raven; 1999.