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(2002) Vitamin D receptor as an intestinal bile acid sensor.
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Makishima M., Lu T.T., Xie W., Whitfield G.K., Domoto H., Evans R.M.et al. (1999) Identification of a nuclear receptor for bile acids.
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Makishima M., Okamoto A.Y., Repa J.J., Tu H., Learned R.M., Luk A.et al. (1999) Bile acids: natural ligands for an orphan nuclear receptor. Parks D.J., Blanchard S.G., Bledsoe R.K., Chandra G., Consler T.G., Kliewer S.A.et al. (2014) Beyond intestinal soap-bile acids in metabolic control. These findings highlight that the potential implications of AB administration to cholestatic patients should be evaluated in a systematic manner.Ĭyp2c70 bile acids gut microbiota hepatobiliary disease hydrophobicity liver fibrosis. In conclusion, depletion of gut microbiota in Cyp2c70-/- mice aggravates liver pathology at least in part by increasing the hydrophobicity of the circulating BA pool. Moreover, genetic inactivation of Cyp8b1 in livers of male Cyp2c70-/- mice increased liver pathology, while addition of ursodeoxycholic acid to the diet prevented weight loss and liver pathology in AB-treated female Cyp2c70-/- mice. Interestingly, the resulting increased BA hydrophobicity strongly correlated with various indicators of liver pathology.
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Dramatic downregulation of hepatic Cyp8b1 expression (-99%) caused a reduction in the proportions of 12α-hydroxylated BAs in the circulating BA pools of AB-treated male Cyp2c70-/- mice. Male Cyp2c70-/- mice did tolerate AB but showed markedly augmented liver pathology after 6 weeks of treatment. Female Cyp2c70-/- mice did not tolerate AB treatment, necessitating premature termination of the experiment. Therefore, we treated young-adult male and female wild-type (WT) and Cyp2c70-/- mice with antibiotics (AB) with broad specificity to deplete the gut microbiota and assessed the consequences on BA metabolism and liver pathology. Although Cyp2c70-ablation has been shown to alter gut microbiome composition, the impact of gut bacteria on liver pathology in Cyp2c70-/- mice remains to be established. However, the hydrophobic BA composition in these animals is associated with liver pathology. LIG4 syndrome see these terms).Cyp2c70-deficient mice have a human-like bile acid (BA) composition due to their inability to convert chenodeoxycholic acid (CDCA) into rodent-specific muricholic acids (MCAs). They may also present with extraimmune manifestations like neurodevelopmental deficit, sensorineural deafness, and hepatic abnormalities (SCID due to adenosine deaminase (ADA) deficiency ) with sensorineural deafness (reticular dysgenesis).Others may show microcephaly with neurodevelopmental delay (e.g. Patients are unable to produce specific antibodies after vaccination or natural infection. SCID due to gamma chain deficiency or SCID due to JAK3 deficiency see this term). Alopecia and skin rash may be present depending on the form (e.g. Patients have an increased susceptibility to opportunistic infections (usually in the respiratory tract and the gut) most often due to P. SCID usually presents within the first few months of life with failure to thrive, severe infections (pneumonia, gastrointestinal infections, sepsis), recurrent or persistent thrush, chronic diarrhea, and/or absent lymph nodes.