Mitochondrial ALA synthase (ALAS)
Cofactors used
Clinical / pharmacological. ALAS2 defects: X-linked sideroblastic anaemia (B6-responsive)
What it does, reaction by reaction
Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol
Glycine + succinyl-CoA → δ-aminolevulinate (ALA) + CO₂ + CoA
Converts Glycine Succinyl-CoA into delta--aminolevulinate (ALA) CO2 CoA-SH
› Notes
Mitochondrial ALA synthase (ALAS) requires PLP (vitamin B6). ALAS1 is the rate-limiting and committed step in nonerythroid cells and is feedback-inhibited at transcriptional, translational, and mitochondrial-import levels by heme/hemin; erythroid ALAS2 is rate-limited by iron-dependent translation through an iron-responsive element.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · from cache · sorted by publication date
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1
SPIC-dependent erythrophagocytic macrophages drive granuloma formation and pathogen persistence during intracellular bacterial infection
· 2026-05-27
open access unreviewed -
2
Imperfect Maturation of Erythroid Progenitors in Patients with Cirrhosis-Associated Anemia.
Lal D, Maras JS, Maiwall R, Kumar A, Bihari C. · 2026-05-14
open access unreviewed -
3
A cell-nonautonomous heme acquisition pathway enables erythroid hemoglobinization under stress.
Belot A, Rock A, Dutt S, Haemmerle G, Maros A, Yuan X, Otsuru S, Bodine D, Hamza I. · 2026-04-23
unreviewed -
4
Unraveling key genes and mitochondrial-related mechanisms of atherosclerosis severity: explorations based on interpretable machine learning.
Kong Z, Zhang XZ, Zhou YY, Wang Q, Pan YH, Lu Y, Ye H, Hong XY. · 2026-02-09
cited 1× open access unreviewed -
5
Mitochondrial Transporter ABCB10 Protects Against Doxorubicin-Induced Respiratory Muscle Dysfunction Independent of Changes to Diaphragm Accumulation.
Smuder AJ, Doerr V, Jacintho Moritz CE, Li J, Nguyen BL. · 2026-02-01
cited 1× open access unreviewed -
6
Targeting Infected Host Cell Heme Metabolism to Kill Malaria Parasites.
Siddiqui FA, Adapa SR, Li X, Miao J, Cui L, Jiang RHY. · 2026-01-17
cited 1× open access unreviewed -
7
The paradoxical prognostic effects of T-cell-derived circulating DNA in EGFR-mutated advanced non-small-cell lung cancer involve the crosstalk between heme biosynthesis and immune microenvironment.
Zungsontiporn N, Ngamchokwathana C, Santisukwongchote S, Sitthideatphaiboon P, Leelayuwatanakul N, Korphaisar… · 2025-10-17
open access unreviewed -
8
TP53 Expression Status Alters Hemoglobinization and Ferroptosis Sensitivity in K-562 Cells.
Cardona C, Young M, Montgomery M. · 2025-08-28
open access unreviewed -
9
From Deficiency to Therapy: Systemic Consequences of ALAS1 Disruption and the Protective Role of 5-ALA.
van Wijk K, Nakajima O. · 2025-08-07
open access unreviewed -
10
Transcriptomics and proteomics reveals the potential mechanisms of hydroquinone-inhibited erythroid differentiation in K562 cells.
Yu C, Chen J, Zhao Z, Tan X, Liu X, Yi Z. · 2025-07-21
unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.