Bile acid analysis in human disorders of bile acid biosynthesis

Molecular Aspects of Medicine

Volumn 56, Issue , 2017, Pages 10-24

  Vaz, Frédéric M ^a   Ferdinandusse, Sacha ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

Bile acid biosynthesis; Inborn errors of metabolism; Mass spectrometry

Indexed keywords

2 METHYLACYL COENZYME A RACEMASE; 3BETA HYDROXY DELTA5 STEROID DEHYDROGENASE; BILE ACID; CHOLESTANETRIOL 26 MONOOXYGENASE; CHOLESTEROL 7ALPHA MONOOXYGENASE; STEROID 5BETA REDUCTASE; CHENODEOXYCHOLIC ACID; CHOLESTEROL; CHOLIC ACID;

3BETA HYDROXY DELTA5 C27 STEROID OXIDOREDUCTASE DEFICIENCY; ACYL COA OXIDASE 2 DEFICIENCY; ALPHA METHYLACYL COA RACEMASE DEFICIENCY; AMIDATION; BILE ACID COA LIGASE DEFICIENCY; BILE ACID METABOLISM; BILE ACID SYNTHESIS; CEREBROTENDINOUS XANTHOMATOSIS; CHOLESTEROL 7ALPHA HYDROXYLASE DEFICIENCY; DELTA BIFUNCTIONAL PROTEIN DEFICIENCY; DELTA4 3 OXOSTEROID 5BETA REDUCTASE DEFICIENCY; DISORDERS OF LIPID AND LIPOPROTEIN METABOLISM; ENTEROHEPATIC CIRCULATION; ENZYME DEFICIENCY; HUMAN; LIPID ANALYSIS; LIQUID CHROMATOGRAPHY-MASS SPECTROMETRY; PROTEIN DEFICIENCY; REVIEW; STEROL 27 HYDROXYLASE DEFICIENCY; STEROL CARRIER PROTEIN X DEFICIENCY; STRUCTURE ANALYSIS; ZELLWEGER SYNDROME; ADULT; ANIMAL; BIOSYNTHESIS; CHOLESTASIS; CYTOLOGY; DEVICES; ENZYMOLOGY; GENETICS; HEREDITARY MOTOR SENSORY NEUROPATHY; HOMEOSTASIS; INFANT; INTESTINE; LIVER; MASS SPECTROMETRY; METABOLISM; MICROBIOLOGY; PATHOLOGY; PHYSIOLOGY; PROCEDURES;

ADULT; ANIMALS; CHENODEOXYCHOLIC ACID; CHOLESTASIS; CHOLESTEROL; CHOLIC ACID; ENTEROHEPATIC CIRCULATION; HOMEOSTASIS; HUMANS; INFANT; INTESTINES; LIVER; MASS SPECTROMETRY; SPASTIC PARAPLEGIA, HEREDITARY; ZELLWEGER SYNDROME;

EID: 85015954225     PISSN: 00982997     EISSN: 18729452     Source Type: Journal    
DOI: 10.1016/j.mam.2017.03.003     Document Type: Review

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