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More than 10<jats:sup>7<\/jats:sup> neutrophils are produced every minute in an adult bone marrow\u2014a process that is tightly regulated by a small group of cytokines and chemical mediators and dependent on nutrients and energy. Like granulocyte colony\u2010stimulating factor, the primary growth factor for granulopoiesis, they stimulate signalling pathways, some affecting metabolism. Nutrient or energy deficiency stresses the survival, proliferation, and differentiation of neutrophils and their precursors. Thus, it is not surprising that monogenic disorders related to metabolism exist that result in neutropenia. Among these are pathogenic mutations in <jats:italic>HAX1<\/jats:italic>, <jats:italic>G6PC3<\/jats:italic>, <jats:italic>SLC37A4<\/jats:italic>, <jats:italic>TAFAZZIN<\/jats:italic>, <jats:italic>SBDS<\/jats:italic>, <jats:italic>EFL1<\/jats:italic> and the mitochondrial disorders. These mutations perturb carbohydrate, lipid and\/or protein metabolism. We hypothesize that metabolic disturbances may drive the pathogenesis of a subset of inherited neutropenias just as defects in DNA damage response do in Fanconi anaemia, telomere maintenance in dyskeratosis congenita and ribosome formation in Diamond\u2013Blackfan anaemia. Greater understanding of metabolic pathways in granulopoiesis will identify points of vulnerability in production and may point to new strategies for the treatment of neutropenias.<\/jats:p>","DOI":"10.1111\/bjh.19192","type":"journal-article","created":{"date-parts":[[2023,12,4]],"date-time":"2023-12-04T20:39:11Z","timestamp":1701722351000},"page":"45-55","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["The metabolic basis of inherited neutropenias"],"prefix":"10.1111","volume":"204","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4082-7945","authenticated-orcid":false,"given":"Usua","family":"Oyarbide","sequence":"first","affiliation":[{"name":"Department of Cancer Biology Cleveland Clinic  Cleveland Ohio USA"},{"name":"Department of Pediatrics Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevieve 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