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The pathway is initiated by the synthesis of [[δ-aminolevulinic acid]] (dALA or δALA) from the [[amino acid]] [[glycine]] and [[succinyl-CoA]] from the [[citric acid cycle]] (Krebs cycle). The rate-limiting enzyme responsible for this reaction, ''ALA synthase'', is negatively regulated by glucose and heme concentration. Mechanism of inhibition of ALAs by heme or hemin is by decreasing stability of mRNA synthesis and by decreasing the intake of mRNA in the mitochondria. This mechanism is of therapeutic importance: infusion of ''heme arginate'' or ''hematin'' and glucose can abort attacks of [[acute intermittent porphyria]] in patients with an [[inborn error of metabolism]] of this process, by reducing transcription of ALA synthase.<ref>{{cite thesis|url=http://escholarship.umassmed.edu/gsbs_diss/121/|title=Upregulation of Heme Pathway Enzyme ALA Synthase-1 by Glutethimide and 4,6-Dioxoheptanoic Acid and Downregulation by Glucose and Heme: A Dissertation|first=Kolluri|last=Sridevi|date=28 April 2018|journal=EScholarship@UMMS|access-date=28 April 2018|url-status=live|archive-url=https://web.archive.org/web/20160808080738/http://escholarship.umassmed.edu/gsbs_diss/121/|archive-date=8 August 2016|doi=10.13028/yyrz-qa79|publisher=University of Massachusetts Medical School}}</ref> |
The pathway is initiated by the synthesis of [[δ-aminolevulinic acid]] (dALA or δALA) from the [[amino acid]] [[glycine]] and [[succinyl-CoA]] from the [[citric acid cycle]] (Krebs cycle). The rate-limiting enzyme responsible for this reaction, ''ALA synthase'', is negatively regulated by glucose and heme concentration. Mechanism of inhibition of ALAs by heme or hemin is by decreasing stability of mRNA synthesis and by decreasing the intake of mRNA in the mitochondria. This mechanism is of therapeutic importance: infusion of ''heme arginate'' or ''hematin'' and glucose can abort attacks of [[acute intermittent porphyria]] in patients with an [[inborn error of metabolism]] of this process, by reducing transcription of ALA synthase.<ref>{{cite thesis|url=http://escholarship.umassmed.edu/gsbs_diss/121/|title=Upregulation of Heme Pathway Enzyme ALA Synthase-1 by Glutethimide and 4,6-Dioxoheptanoic Acid and Downregulation by Glucose and Heme: A Dissertation|first=Kolluri|last=Sridevi|date=28 April 2018|journal=EScholarship@UMMS|access-date=28 April 2018|url-status=live|archive-url=https://web.archive.org/web/20160808080738/http://escholarship.umassmed.edu/gsbs_diss/121/|archive-date=8 August 2016|doi=10.13028/yyrz-qa79|publisher=University of Massachusetts Medical School}}</ref> |
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The organs mainly involved in heme synthesis are the [[liver]] (in which the rate of synthesis is highly variable, depending on the systemic heme pool) and the [[bone marrow]] (in which rate of synthesis of Heme is relatively constant and depends on the production of globin chain), although every cell requires heme to function properly. However, due to its toxic properties, proteins such as [[emopexin]] (Hx) are required to help maintain physiological stores of iron in order for them to be used in synthesis.<ref name="ReferenceA">{{cite journal|last1=Kumar|first1=Sanjay|last2=Bandyopadhyay|first2=Uday|title=Free heme toxicity and its detoxification systems in human|journal=Toxicology Letters|date=July 2005|volume=157|issue=3|pages=175–188|doi=10.1016/j.toxlet.2005.03.004|pmid=15917143}}</ref> Heme is seen as an intermediate molecule in catabolism of hemoglobin in the process of [[bilirubin metabolism]]. Defects in various enzymes in synthesis of heme can lead to group of disorder called porphyrias, which include [[acute intermittent porphyria]], [[congenital erythropoetic porphyria]], [[porphyria cutanea tarda]], [[hereditary coproporphyria]], [[variegate porphyria]], and [[erythropoietic protoporphyria]].<ref>{{Cite journal|last1=Puy|first1=Hervé|last2=Gouya|first2=Laurent|last3=Deybach|first3=Jean-Charles|date=March 2010|title=Porphyrias|url=https://linkinghub.elsevier.com/retrieve/pii/S0140673609619255|journal=The Lancet|language=en|volume=375|issue=9718|pages=924–937|doi=10.1016/S0140-6736(09)61925-5|pmid=20226990|s2cid=208791867}}</ref> |
The organs mainly involved in heme synthesis are the [[liver]] (in which the rate of synthesis is highly variable, depending on the systemic heme pool) and the [[bone marrow]] (in which rate of synthesis of Heme is relatively constant and depends on the production of globin chain), although every cell requires heme to function properly. However, due to its toxic properties, proteins such as [[emopexin]] (Hx) are required to help maintain physiological stores of iron in order for them to be used in synthesis.<ref name="ReferenceA">{{cite journal|last1=Kumar|first1=Sanjay|last2=Bandyopadhyay|first2=Uday|title=Free heme toxicity and its detoxification systems in human|journal=Toxicology Letters|date=July 2005|volume=157|issue=3|pages=175–188|doi=10.1016/j.toxlet.2005.03.004|pmid=15917143}}</ref> Heme is seen as an intermediate molecule in catabolism of hemoglobin in the process of [[bilirubin metabolism]]. Defects in various enzymes in synthesis of heme can lead to group of disorder called porphyrias, which include [[acute intermittent porphyria]], [[congenital erythropoetic porphyria]], [[porphyria cutanea tarda]], [[hereditary coproporphyria]], [[variegate porphyria]], and [[erythropoietic protoporphyria]].<ref>{{Cite journal|last1=Puy|first1=Hervé|last2=Gouya|first2=Laurent|last3=Deybach|first3=Jean-Charles|date=March 2010|title=Porphyrias|url=https://linkinghub.elsevier.com/retrieve/pii/S0140673609619255|journal=The Lancet|language=en|volume=375|issue=9718|pages=924–937|doi=10.1016/S0140-6736(09)61925-5|pmid=20226990|s2cid=208791867}}</ref>{{Citation needed|date=December 2016}} |
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==Synthesis for food== |
==Synthesis for food== |
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