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Select item 14589841.

GeneReviews® [Internet].

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].

StatPearls [Internet].

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

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Select item 44650643.

Autoimmunity: From Bench to Bedside [Internet].

Autoimmunity: From Bench to Bedside [Internet].

Anaya JM, Shoenfeld Y, Rojas-Villarraga A, et al., editors.

Bogota (Colombia): El Rosario University Press; 2013 Jul 18.

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Select item 14152804.

Madame Curie Bioscience Database [Internet].

Madame Curie Bioscience Database [Internet].

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

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Select item 18813825.

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

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 42037206.

Streptococcus pyogenes : Basic Biology to Clinical Manifestations [Internet].

Streptococcus pyogenes : Basic Biology to Clinical Manifestations [Internet].

Ferretti JJ, Stevens DL, Fischetti VA, editors.

Oklahoma City (OK): University of Oklahoma Health Sciences Center; 2016-.

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Select item 54017687.

Streptococcus pyogenes: Basic Biology to Clinical Manifestations [Internet]. 2nd edition.

Streptococcus pyogenes: Basic Biology to Clinical Manifestations [Internet]. 2nd edition.

Ferretti JJ, Stevens DL, Fischetti VA, editors.

Oklahoma City (OK): University of Oklahoma Health Sciences Center; 2022 Oct 8.

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Select item 52412088.

The Heterogeneity of Cancer Metabolism [Internet]. 2nd edition.

The Heterogeneity of Cancer Metabolism [Internet]. 2nd edition.

Le A, editor.

Cham (CH): Springer; 2021.

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Select item 49585449.

Ratio of phosphate to amino acids: Neonatal parenteral nutrition: Evidence review D10.

Ratio of phosphate to amino acids: Neonatal parenteral nutrition: Evidence review D10.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495848010.

Ratio of calcium to phosphate: Neonatal parenteral nutrition: Evidence review D9.

Ratio of calcium to phosphate: Neonatal parenteral nutrition: Evidence review D9.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495825411.

Lipids: Neonatal parenteral nutrition: Evidence review D3.

Lipids: Neonatal parenteral nutrition: Evidence review D3.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495818412.

Ratio of carbohydrates to lipids: Neonatal parenteral nutrition: Evidence review D8.

Ratio of carbohydrates to lipids: Neonatal parenteral nutrition: Evidence review D8.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495811813.

Ratio of non-nitrogen energy to nitrogen: Neonatal parenteral nutrition: Evidence review D7.

Ratio of non-nitrogen energy to nitrogen: Neonatal parenteral nutrition: Evidence review D7.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495801414.

Glucose: Neonatal parenteral nutrition: Evidence review D1.

Glucose: Neonatal parenteral nutrition: Evidence review D1.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 495797015.

Amino acids: Neonatal parenteral nutrition: Evidence review D2.

Amino acids: Neonatal parenteral nutrition: Evidence review D2.

National Guideline Alliance (UK).

London: National Institute for Health and Care Excellence (NICE); 2020 Feb. (NICE Guideline, No. 154.)

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Select item 449545016.

Glycerol Phenylbutyrate (Ravicti) [Internet].

Glycerol Phenylbutyrate (Ravicti) [Internet].

Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2017 Apr.

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Select item 235805217.

Probe Reports from the NIH Molecular Libraries Program [Internet].

Probe Reports from the NIH Molecular Libraries Program [Internet].

Bethesda (MD): National Center for Biotechnology Information (US); 2010-.

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Select item 166869018.

Holland-Frei Cancer Medicine. 6th edition.

Holland-Frei Cancer Medicine. 6th edition.

Kufe DW, Pollock RE, Weichselbaum RR, et al., editors.

Hamilton (ON): BC Decker; 2003.

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Select item 553690419.

Dietary Protein Intake: A Series of Evidence Scans on Acute Adverse Health Effects, Chronic Disease Risk, and Daily Requirements [Internet].

Dietary Protein Intake: A Series of Evidence Scans on Acute Adverse Health Effects, Chronic Disease Risk, and Daily Requirements [Internet].

Spahn J, Bahnfleth C, Bates M, et al.

Alexandria (VA): USDA Nutrition Evidence Systematic Review; 2022 Mar.

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Select item 425931420.

Reproductive, Maternal, Newborn, and Child Health: Disease Control Priorities, Third Edition (Volume 2).

Reproductive, Maternal, Newborn, and Child Health: Disease Control Priorities, Third Edition (Volume 2).

Black RE, Laxminarayan R, Temmerman M, et al., editors.

Washington (DC): The International Bank for Reconstruction and Development / The World Bank; 2016 Apr 5.

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Supplemental Content

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Figure 7:

Schematic diagram showing the arginine and citrulline catabolic pathway in S. pyogenes and its coordination with F₁F₀-ATPases. Transport and catabolism of arginine and citrulline in S. pyogenes involve a multienzyme arginine deiminase pathway, wherein ArcD serves as an antiporter and/or an unknown transporter. The transported arginine is then catabolized via the enzymes ArcA, ArcB, and ArcC, which results in two molecules of ammonia and one molecule of ATP. Catabolism of citrulline, on the other hand, produces one molecule each of ammonia and ATP. F₁F₀-ATPase then exports three protons outside the cell with simultaneous hydrolysis of ATP to ADP. The Spy_xxxx assignments are based on the strain type M1 S. pyogenes MGAS5005 genome.

Streptococcus pyogenes Metabolism.

Ferretti JJ, Stevens DL, Fischetti VA, editors. Streptococcus pyogenes: Basic Biology to Clinical Manifestations [Internet]. 2nd edition. Oklahoma City (OK): University of Oklahoma Health Sciences Center; 2022 Oct 8.
Figure 7:

Schematic diagram showing the arginine and citrulline catabolic pathway in S. pyogenes and its coordination with F₁F₀-ATPases. Transport and catabolism of arginine and citrulline in S. pyogenes involve a multienzyme arginine deiminase pathway, wherein ArcD serves as an antiporter and/or an unknown transporter. The transported arginine is then catabolized via the enzymes ArcA, ArcB, and ArcC, which results in two molecules of ammonia and one molecule of ATP. Catabolism of citrulline, on the other hand, produces one molecule each of ammonia and ATP. F₁F₀-ATPase then exports three protons outside the cell with simultaneous hydrolysis of ATP to ADP. The Spy_xxxx assignments are based on the strain type M1 S. pyogenes MGAS5005 genome.

Streptococcus pyogenes Metabolism.

Ferretti JJ, Stevens DL, Fischetti VA, editors. Streptococcus pyogenes : Basic Biology to Clinical Manifestations [Internet]. Oklahoma City (OK): University of Oklahoma Health Sciences Center; 2016-.
Figure 1.

Clinical and laboratory manifestations of citrin deficiency
AA = amino acids; AFP = alpha-fetoprotein; Arg = arginine; Cit = citrulline; CTLN2 = type II citrullinemia; FTTDCD = failure to thrive and dyslipidemia caused by citrin deficiency; Met = methionine; NICCD = neonatal intrahepatic cholestasis caused by citrin deficiency; Thr = threonine; Tyr = tyrosine

Citrin Deficiency.

Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2025.
FIGURE 24

Endothelial hyperpermeability signaling through nitric oxide. Activation of endothelial nitric oxide synthase (eNOS) by Ca²⁺ binding or through phosphorylation of serine (S) 1179, catalyzes the conversion of L-arginine to citrulline and nitric oxide (NO). Under inflammatory conditions, eNOS activation may occur in response to Src-mediated phosphorylation of caveolin-1, and subsequent release of sequestered eNOS from caveolae. NO production causes microvascular endothelial hyperpermeability by combining with superoxide-free radicals to form peroxynitrate (ONOO⁻) and impairing mitochondrial function. NO also activates guanylate cyclase (GC), which converts GTP to cGMP and activates protein kinase G (PKG). PKG activates the Raf-MEK-ERK signal cascade and causes endothelial hyperpermeability through increased actomyosin contraction.

Signaling Mechanisms in the Regulation of Endothelial Permeability.

Yuan SY, Rigor RR. Regulation of Endothelial Barrier Function. San Rafael (CA): Morgan & Claypool Life Sciences; 2010.

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