Cholate-CoA ligase (,EEL) and bile acid-CoA: amino acid N-acyltransferase (BAAT) sequentially mediate bile-acid amidation. Defects can cause intrahepatic cholestasis. Distinction has required gene sequencing. We ...Cholate-CoA ligase (,EEL) and bile acid-CoA: amino acid N-acyltransferase (BAAT) sequentially mediate bile-acid amidation. Defects can cause intrahepatic cholestasis. Distinction has required gene sequencing. We assessed potential clinical utility of immunostaining of liver for CCL and BAAT. Using commercially available antibodies against BAAT and CCL, we immunostained liver from an infant with jaundice, deficiency of amidated bile acids, and transcription-terminating mutation in BAAT. CCL was normally expressed. BAAT expression was not de- tected. Immunostaining may facilitate diagnosis in bile- acid amidation defects.展开更多
Due to the important role of vitamin D in metabolism,developing a method for rapid and high-throughput analysis of vitamin D and its metabolites is crucial.However,the current clinical diagnostic methods have the disa...Due to the important role of vitamin D in metabolism,developing a method for rapid and high-throughput analysis of vitamin D and its metabolites is crucial.However,the current clinical diagnostic methods have the disadvantage of complicated operating procedures.In the present study,a technique combining immunocapture with mass spectrometry for rapidly detecting of 25-hydroxyvitamin D_(3)in serum was developed.This method employs an antibody–antigen reaction to specifically enrich 25-hydroxyvitamin D_(3)and remove complex interfering substances.Qualitative and quantitative analysis of 25-hydroxyvitamin D_(3)was achieved by nano-electrospray ionization-mass spectrometry(nano-ESI–MS).The method exhibited good linearity in the range of 1–100 ng/mL with satisfactory recovery and repeatability.The proposed method is promising for high-throughput and rapid analysis of small molecules with mass spectrometry.And it can potentially be combined with portable mass spectrometers to meet the requirements of point-of-care detection.展开更多
基金Supported by Great Ormond Street Hospital Children’s Charity, to Clayton PTNational Institutes of HealthGrant R01 DK58214, to Bull LN
文摘Cholate-CoA ligase (,EEL) and bile acid-CoA: amino acid N-acyltransferase (BAAT) sequentially mediate bile-acid amidation. Defects can cause intrahepatic cholestasis. Distinction has required gene sequencing. We assessed potential clinical utility of immunostaining of liver for CCL and BAAT. Using commercially available antibodies against BAAT and CCL, we immunostained liver from an infant with jaundice, deficiency of amidated bile acids, and transcription-terminating mutation in BAAT. CCL was normally expressed. BAAT expression was not de- tected. Immunostaining may facilitate diagnosis in bile- acid amidation defects.
基金supported by the NationalNatural Science Foundation of China(21927808,21974078,21727813,and 22227803)the China Ministry of Science and Technologyunder Contract(2022YFF0710200)。
文摘Due to the important role of vitamin D in metabolism,developing a method for rapid and high-throughput analysis of vitamin D and its metabolites is crucial.However,the current clinical diagnostic methods have the disadvantage of complicated operating procedures.In the present study,a technique combining immunocapture with mass spectrometry for rapidly detecting of 25-hydroxyvitamin D_(3)in serum was developed.This method employs an antibody–antigen reaction to specifically enrich 25-hydroxyvitamin D_(3)and remove complex interfering substances.Qualitative and quantitative analysis of 25-hydroxyvitamin D_(3)was achieved by nano-electrospray ionization-mass spectrometry(nano-ESI–MS).The method exhibited good linearity in the range of 1–100 ng/mL with satisfactory recovery and repeatability.The proposed method is promising for high-throughput and rapid analysis of small molecules with mass spectrometry.And it can potentially be combined with portable mass spectrometers to meet the requirements of point-of-care detection.