PMID: 2492704Feb 1, 1989Paper

Gonadal function and ovarian galactose metabolism in classic galactosemia

Acta Endocrinologica
F R KaufmanG N Donnell

Abstract

Evaluation of ovarian steroid secretion, histologic examination of ovarian tissue, and incubation studies with radiolabelled galactose in ovarian tissue slices were performed in a 21-year-old woman with galactosemia and incipient ovarian failure. After exogenous gonadotropin administration in an attempt to achieve fertility, there was no evidence of ovulation by ultrasound; estrogen and androgen production were deficient indicating ovarian unresponsiveness. Histologic examination of the ovary revealed that the ovarian stroma had an increase in fibrous tissue and that a few hyalinized atretic follicles were present with no intermediate or evolving Graafian follicles. After incubation with galactose-1-14C, there was absence of labelled CO2 production and only labelled galactose-1-phosphate was identified as compared to controls in which several labelled intermediates could be seen. The incorporation of galactose into the TCA-insoluble fraction was drastically reduced in the patient compared to controls, suggesting that there may be a deficiency of ovarian galactose-containing glycolipids, glycoproteins and mucopolysaccharides in the galactosemic ovary. Deficiency in the production of galactose containing compounds, or galactose-1...Continue Reading

Citations

Jan 1, 1990·Journal of Inherited Metabolic Disease·R G WilsonP E Hartmann
Jan 1, 1990·Journal of Inherited Metabolic Disease·S Segal, S Rogers
May 4, 2005·Fertility and Sterility·Enver SimsekJohn C Achermann
Oct 6, 2000·Reproductive Toxicology·G LiuC L Hughes
Apr 12, 2003·Pacing and Clinical Electrophysiology : PACE·David O'DonnellSteve S Furniss
Jan 11, 1992·Journal of Inherited Metabolic Disease·H N Kirkman
Jan 1, 1995·European Journal of Pediatrics·J B Gibson
Jun 25, 2008·Annals of the New York Academy of Sciences·Gerard T Berry
Oct 2, 2009·Human Reproduction Update·M E Rubio-GozalboJ A Land
Oct 28, 2009·IUBMB Life·Kent LaiKlaas J Wierenga
Jul 13, 2006·Human Reproduction Update·T ForgesP Jouvet
Mar 4, 1999·The New England Journal of Medicine·E Y Adashi, J D Hennebold
Nov 10, 2001·Annual Review of Genomics and Human Genetics·H L Levy, S Albers
Apr 22, 2003·Annual Review of Nutrition·Nancy D Leslie
Sep 22, 2017·Journal of Assisted Reproduction and Genetics·Mili ThakurElizabeth E Puscheck
May 29, 2018·Journal of Assisted Reproduction and Genetics·Linn Salto MamsenClaus Yding Andersen
Jan 6, 2009·Bioscience Hypotheses·K LaiLj Elsas
Apr 13, 2010·Reproductive Biomedicine Online·Hridesh DixitLalji Singh

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