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The patients repeated the submaximal exercise after 2\u2009days, where they received a 10% iv\u2010glucose supplementation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients had lower VO<jats:sub>2max<\/jats:sub> than healthy controls, and two of three patients had to stop prematurely during the intended 1\u2010hour submaximal exercise test. During nonischemic forearm test, all patients were able to produce lactate in normal amounts. Glucose infusion had no effect on patients' exercise capacity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with GSDXIII experience exercise intolerance and episodes of myoglobinuria, even to the point of needing renal dialysis, but still retain an almost normal anaerobic metabolic response to submaximal intensity exercise. In accordance with this, glucose supplementation did not improve exercise capacity. The findings show that GSDXIII, although causing episodic rhabdomyolysis, is one of the mildest metabolic myopathies affecting glycolysis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jmd2.12232","type":"journal-article","created":{"date-parts":[[2021,6,15]],"date-time":"2021-06-15T02:26:06Z","timestamp":1623723966000},"page":"60-66","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Energy metabolism during exercise in patients with \u03b2\u2010enolase deficiency (<scp>GSDXIII<\/scp>)"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8571-5480","authenticated-orcid":false,"given":"Astrid Emilie","family":"Buch","sequence":"first","affiliation":[{"name":"Copenhagen Neuromuscular Center, Rigshospitalet, University of Copenhagen  Copenhagen 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