PMID- 29873499
OWN - NLM
STAT- MEDLINE
DCOM- 20190610
LR  - 20190613
IS  - 1535-3907 (Electronic)
IS  - 1535-3893 (Linking)
VI  - 17
IP  - 7
DP  - 2018 Jul 6
TI  - Metabolic Profiling of Multiorgan Samples: Evaluation of MODY5/RCAD Mutant Mice.
PG  - 2293-2306
LID - 10.1021/acs.jproteome.7b00821 [doi]
AB  - In the present study, we performed a metabolomics analysis to evaluate a 
      MODY5/RCAD mouse mutant line as a potential model for HNF1B-associated diseases. 
      Gas chromatography time-of-flight mass spectrometry (GC-TOF-MS) of gut, kidney, 
      liver, muscle, pancreas, and plasma samples uncovered the tissue specific 
      metabolite distribution. Orthogonal projections to latent structures discriminant 
      analysis (OPLS-DA) was used to identify the differences between MODY5/RCAD and 
      wild-type mice in each of the tissues. The differences included, for example, 
      increased levels of amino acids in the kidneys and reduced levels of fatty acids 
      in the muscles of the MODY5/RCAD mice. Interestingly, campesterol was found in 
      higher concentrations in the MODY5/RCAD mice, with a four-fold and three-fold 
      increase in kidneys and pancreas, respectively. As expected, the MODY5/RCAD mice 
      displayed signs of impaired renal function in addition to disturbed liver lipid 
      metabolism, with increased lipid and fatty acid accumulation in the liver. From a 
      metabolomics perspective, the MODY5/RCAD model was proven to display a metabolic 
      pattern similar to what would be suspected in HNF1B-associated diseases. These 
      findings were in line with the presumed outcome of the mutation based on the 
      different anatomy and function of the tissues as well as the effect of the 
      mutation on development.
FAU - Torell, Frida
AU  - Torell F
AUID- ORCID: 0000-0002-6294-7844
AD  - Computational Life Science Cluster (CLiC), Department of Chemistry , Umea 
      University , Umea 90187 , Sweden.
AD  - Accelerator Lab (ACL) , Karlsruhe Institute of Technology , Karlsruhe 76344 , 
      Germany.
FAU - Bennett, Kate
AU  - Bennett K
AD  - AcureOmics AB, Umea 90736 , Sweden.
FAU - Cereghini, Silvia
AU  - Cereghini S
AD  - CNRS, UMR7622, 75005 Paris , France.
AD  - UPMC, UMR7622 , Sorbonne Universites , 75005 Paris , France.
AD  - Inserm U-1156 Paris , France.
FAU - Fabre, Melanie
AU  - Fabre M
AD  - CNRS, UMR7622, 75005 Paris , France.
AD  - UPMC, UMR7622 , Sorbonne Universites , 75005 Paris , France.
AD  - Inserm U-1156 Paris , France.
FAU - Rannar, Stefan
AU  - Rannar S
AD  - AcureOmics AB, Umea 90736 , Sweden.
FAU - Lundstedt-Enkel, Katrin
AU  - Lundstedt-Enkel K
AD  - AcureOmics AB, Umea 90736 , Sweden.
AD  - Department of Organismal Biology , Uppsala University , Uppsala 75236 , Sweden.
FAU - Moritz, Thomas
AU  - Moritz T
AD  - AcureOmics AB, Umea 90736 , Sweden.
AD  - Umea Plant Science Centre, Department of Forest Genetics and Plant Physiology , 
      Swedish University of Agricultural Sciences , Umea 901 87 , Sweden.
FAU - Haumaitre, Cecile
AU  - Haumaitre C
AD  - CNRS, UMR7622, 75005 Paris , France.
AD  - UPMC, UMR7622 , Sorbonne Universites , 75005 Paris , France.
AD  - Inserm U-1156 Paris , France.
FAU - Trygg, Johan
AU  - Trygg J
AUID- ORCID: 0000-0003-3799-6094
AD  - Computational Life Science Cluster (CLiC), Department of Chemistry , Umea 
      University , Umea 90187 , Sweden.
FAU - Lundstedt, Torbjorn
AU  - Lundstedt T
AD  - AcureOmics AB, Umea 90736 , Sweden.
AD  - Department of Organic Pharmaceutical Chemistry , Uppsala University , Uppsala 
      75123 , Sweden.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20180615
PL  - United States
TA  - J Proteome Res
JT  - Journal of proteome research
JID - 101128775
RN  - 0 (Cadherins)
RN  - 0 (R-cadherin)
RN  - 138674-15-4 (Hepatocyte Nuclear Factor 1-beta)
SB  - IM
MH  - Animals
MH  - Cadherins/genetics
MH  - *Disease Models, Animal
MH  - Gas Chromatography-Mass Spectrometry
MH  - Hepatocyte Nuclear Factor 1-beta/genetics
MH  - Kidney/metabolism
MH  - Liver/metabolism
MH  - Metabolomics/*methods
MH  - Mice
MH  - Mice, Mutant Strains/*metabolism
MH  - Pancreas/metabolism
OTO - NOTNLM
OT  - HNF1B-associated diseases
OT  - MODY5
OT  - OPLS-DA
OT  - RCAD
OT  - metabolomics
OT  - mouse model
OT  - multiorgan samples
EDAT- 2018/06/07 06:00
MHDA- 2019/06/14 06:00
CRDT- 2018/06/07 06:00
PHST- 2018/06/07 06:00 [pubmed]
PHST- 2019/06/14 06:00 [medline]
PHST- 2018/06/07 06:00 [entrez]
AID - 10.1021/acs.jproteome.7b00821 [doi]
PST - ppublish
SO  - J Proteome Res. 2018 Jul 6;17(7):2293-2306. doi: 10.1021/acs.jproteome.7b00821. 
      Epub 2018 Jun 15.