Philipp Christen . Rolf Jaussi . Roger Benoit

	Metabolism of proteins (reactome.org) The discovery of lysosomes and autophagy (Castro-Obregon, S. 2010, Nature Education 3(9):49): Artikel zur Entdeckung der Lysosomen und Autophagie QF-DE of lysosome lumens show paracrystalline sheets (R. Allen, www5.pbrc.hawaii.edu): Gefrierätz-EM-Bild der Lysosomen von Paramecium multimicronucleatum Deep classification of a large cryo-EM dataset defines the conformational landscape of the 26S proteasome (Unverdorben et al. 2014, Proc Natl Acad Sci USA 111: 5544, RCSB PDB): Struktur des 26S Proteasoms

18.2 Abbau der Aminosäuren: Weg des Stickstoffs

Introduction to nitrogen metabolism (the medicalbiochemistrypage.org): Stickstoffmetabolismus

Metabolism of amino acids and derivatives (reactome.org)

Nitrogen/amino acid metabolism I (OpenCourseWare, https://ocw.mit.edu/courses/, Prof. M.V. Heiden)

	Introduction to nitrogen metabolism (the medicalbiochemistrypage.org): Stickstoffmetabolismus Metabolism of amino acids and derivatives (reactome.org) Nitrogen/amino acid metabolism I (OpenCourseWare, https://ocw.mit.edu/courses/, Prof. M.V. Heiden)

18.3 Abbau der Aminosäuren: Weg des Kohlenstoffs

Phenylalanine hydroxylase (David S. Goodsell, RCSB PDB): Struktur und Funktion der Phenylalaninhydroxylase

Butterfly dyes (wildflowerfinder.org.uk): Informationen zu verschiedenen Pterin-Verbindungen

Pterine und Tetrahydrobiopterin

Albinism (www.dnatube.com): Video zum Albinismus

Tyrosinase (YouTube): Animation zu Albinismus, Tyrosinase, Melaninsynthese, UV-Schutz, Braunfärbung pflanzlicher Nahrungsmittel

Understanding UVA and UVB (www.skincancer.org): Informationen zu UVA- und UVB-Strahlen

Polyamine (Wikipedia)

Tyrosinase (Wikipedia)

Inhibition of tyrosinase-induced enzymatic browning by sulfite and natural alternatives (Kuijpers): Hemmung der durch Tyrosinaseaktivität verursachten Braunfärbung pflanzlicher Nahrungsmittel

	Phenylalanine hydroxylase (David S. Goodsell, RCSB PDB): Struktur und Funktion der Phenylalaninhydroxylase Butterfly dyes (wildflowerfinder.org.uk): Informationen zu verschiedenen Pterin-Verbindungen Pterine und Tetrahydrobiopterin Albinism (www.dnatube.com): Video zum Albinismus Tyrosinase (YouTube): Animation zu Albinismus, Tyrosinase, Melaninsynthese, UV-Schutz, Braunfärbung pflanzlicher Nahrungsmittel Understanding UVA and UVB (www.skincancer.org): Informationen zu UVA- und UVB-Strahlen Polyamine (Wikipedia) Tyrosinase (Wikipedia) Inhibition of tyrosinase-induced enzymatic browning by sulfite and natural alternatives (Kuijpers): Hemmung der durch Tyrosinaseaktivität verursachten Braunfärbung pflanzlicher Nahrungsmittel

18.4 Störungen im Abbau von Aminosäuren

What is alkaptonuria (AKU)? (YouTube, Patrick Richter): Vorlesung zur Alkaptonurie

The incidence of alkaptonuria: A study in chemical individuality (Electronic scholarly publishing, www.esp.org, A.E. Garrod): Archibald E. Garrod zum Vorkommen von Alkaptonurie

What are common treatments for phenylketonuria (PKU)? (nichd.nih.gov): Kurzübersicht zu Behandlungsmethoden von Phenylketonurie

	What is alkaptonuria (AKU)? (YouTube, Patrick Richter): Vorlesung zur Alkaptonurie The incidence of alkaptonuria: A study in chemical individuality (Electronic scholarly publishing, www.esp.org, A.E. Garrod): Archibald E. Garrod zum Vorkommen von Alkaptonurie What are common treatments for phenylketonuria (PKU)? (nichd.nih.gov): Kurzübersicht zu Behandlungsmethoden von Phenylketonurie

18.5 Synthese der Aminosäuren

An evolutionary perspective on amino acids (Gutiérrez-Preciado et al. 2010, Nature Education, 3(9):29): Evolution der Biosynthese von Aminosäuren

Introduction to amino acid metabolism (Michael W. King, themedicalbiochemistrypage.org): Einführung zum Metabolismus der Aminosäuren

	An evolutionary perspective on amino acids (Gutiérrez-Preciado et al. 2010, Nature Education, 3(9):29): Evolution der Biosynthese von Aminosäuren Introduction to amino acid metabolism (Michael W. King, themedicalbiochemistrypage.org): Einführung zum Metabolismus der Aminosäuren

18.6 C₁-Stoffwechsel

One-carbon metabolism (Homo sapiens, WikiPathways Adriaens et al.): C₁-Stoffwechsel

Fluorouracil (chemocare.com)

Struktur und Funktion von Folsäure-Antagonisten

	One-carbon metabolism (Homo sapiens, WikiPathways Adriaens et al.): C₁-Stoffwechsel Fluorouracil (chemocare.com) Struktur und Funktion von Folsäure-Antagonisten

18.7 Synthese von Kreatin und Porphyrinen aus Aminosäuren

Schweizerische Gesellschaft für Porphyrie (www.porphyria.ch)

Porphyrie (Wikipedia)

Metabolism of porphyrins (reactome.org)

	Schweizerische Gesellschaft für Porphyrie (www.porphyria.ch) Porphyrie (Wikipedia) Metabolism of porphyrins (reactome.org)

Weiterführende Literatur

	Ling et al. 2023, Signal Transduct Target Ther - "Amino acid metabolism in health and disease" Belot et al. 2024, Liver Int. - "Update on heme biosynthesis, tissue-specific regulation, heme transport, relation to iron metabolism and cellular energy" Clare et al. 2019, Annu Rev Anim Biosci - "One-Carbon Metabolism: Linking Nutritional Biochemistry to Epigenetic Programming of Long-Term Development" White et al. 2025, Nat Rev Endocrinol - "Islet hormones at the intersection of glucose and amino acid metabolism" Maxwell et al. 2021, Science - "Ubiquitination is essential for recovery of cellular activities after heat shock" Wang and Zhang 2020, Nature - "Molecules in the blood of older people promote cancer spread" Makino et al. 2013, Nat Rev Mol Cell Biol - "The RNA exosome and proteasome: common principles of degradation control" Adams 2003, Cancer Treat Rev - "The proteasome: structure, function, and role in the cell" Jung and Grune 2013, Redox Biol - "The proteasome and the degradation of oxidized proteins: Part I-structure of proteasomes" Nixon 2013, Nat Med - "The role of autophagy in neurodegenerative disease" Zhou 2004, Methods Mol Biol - "Determining protein half-lives" Toyama and Hetzer 2013, Nat Rev Mol Cell Biol - "Protein homeostasis: live long, won't prosper" Mendes et al. 2014, Expert Opin Drug Discov - "Design and discovery of mushroom tyrosinase inhibitors and their therapeutic applications" Erlandsen and Stevens 1999, Mol Genet Metab - "The structural basis of phenylketonuria" Wijnen et al. 2007, J Insect Physiol - "Colors and pterin pigmentation of pierid butterfly wings" Flydal and Martinez 2013, IUBMB Life - "Phenylalanine hydroxylase: function, structure, and regulation" Rush et al. 2014, Eur J Clin Nutr - "Vitamin B12: one carbon metabolism, fetal growth and programming for chronic disease" Mogk et al. 2007, Trends Cell Biol - "The N-end rule pathway for regulated proteolysis: prokaryotic and eukaryotic strategies" Varshavsky 2005, Trends Biochem Sci - "Regulated protein degradation" Randhawa et al. 2014, PLoS One - "Metabolic signature of sun exposed skin suggests catabolic pathway overweighs anabolic pathway" Brenner and Hearing 2008, Photochem Photobiol - "The protective role of melanin against UV damage in human skin" Pegg 2009, IUBMB Life - "Mammalian polyamine metabolism and function" Revision September 2026

Ling et al. 2023, Signal Transduct Target Ther - "Amino acid metabolism in health and disease"

Belot et al. 2024, Liver Int. - "Update on heme biosynthesis, tissue-specific regulation, heme transport, relation to iron metabolism and cellular energy"

Clare et al. 2019, Annu Rev Anim Biosci - "One-Carbon Metabolism: Linking Nutritional Biochemistry to Epigenetic Programming of Long-Term Development"

White et al. 2025, Nat Rev Endocrinol - "Islet hormones at the intersection of glucose and amino acid metabolism"

Maxwell et al. 2021, Science - "Ubiquitination is essential for recovery of cellular activities after heat shock"

Wang and Zhang 2020, Nature - "Molecules in the blood of older people promote cancer spread"

Makino et al. 2013, Nat Rev Mol Cell Biol - "The RNA exosome and proteasome: common principles of degradation control"

Adams 2003, Cancer Treat Rev - "The proteasome: structure, function, and role in the cell"

Jung and Grune 2013, Redox Biol - "The proteasome and the degradation of oxidized proteins: Part I-structure of proteasomes"

Nixon 2013, Nat Med - "The role of autophagy in neurodegenerative disease"

Zhou 2004, Methods Mol Biol - "Determining protein half-lives"

Toyama and Hetzer 2013, Nat Rev Mol Cell Biol - "Protein homeostasis: live long, won't prosper"