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Conditionally Immortal Slc4a11-/- Mouse Corneal Endothelial Cell Line Recapitulates Disrupted Glutaminolysis Seen in Slc4a11-/- Mouse Model.

Abstract To establish conditionally immortal mouse corneal endothelial cell lines with genetically matched Slc4a11+/+ and Slc4a11-/- mice as a model for investigating pathology and therapies for SLC4A11 associated congenital hereditary endothelial dystrophy (CHED) and Fuchs' endothelial corneal dystrophy.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title investigative ophthalmology & visual science
Publication Year Start




PMID- 28738416
OWN - NLM
STAT- MEDLINE
DA  - 20170724
DCOM- 20170728
LR  - 20170728
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Linking)
VI  - 58
IP  - 9
DP  - 2017 Jul 01
TI  - Conditionally Immortal Slc4a11-/- Mouse Corneal Endothelial Cell Line
      Recapitulates Disrupted Glutaminolysis Seen in Slc4a11-/- Mouse Model.
PG  - 3723-3731
LID - 10.1167/iovs.17-21781 [doi]
AB  - Purpose: To establish conditionally immortal mouse corneal endothelial cell lines
      with genetically matched Slc4a11+/+ and Slc4a11-/- mice as a model for
      investigating pathology and therapies for SLC4A11 associated congenital
      hereditary endothelial dystrophy (CHED) and Fuchs' endothelial corneal dystrophy.
      Methods: We intercrossed H-2Kb-tsA58 mice (Immortomouse) expressing an IFN-gamma 
      dependent and temperature-sensitive mutant of the SV40 large T antigen (tsTAg)
      with Slc4a11+/+ and Slc4a11-/- C57BL/6 mice. The growth characteristics of the
      cell lines was assessed by doubling time. Ion transport activities (Na+/H+
      exchange, bicarbonate, lactate, and Slc4a11 ammonia transport) were analyzed by
      intracellular pH measurement. The metabolic status of the cell lines was assessed
      by analyzing TCA cycle intermediates via gas chromatography mass spectrometry
      (GC-MS). Results: The immortalized Slc4a11+/+ and Slc4a11-/- mouse corneal
      endothelial cells (MCECs) remained proliferative through passage 49 and
      maintained similar active ion transport activity. As expected, proliferation was 
      temperature sensitive and IFN-gamma dependent. Slc4a11-/- MCECs exhibited
      decreased proliferative capacity, reduced NH3:H+ transport, altered expression of
      glutaminolysis enzymes similar to the Slc4a11-/- mouse, and reduced proportion of
      TCA cycle intermediates derived from glutamine with compensatory increases in
      glucose flux compared with Slc4a11+/+ MCECs. Conclusions: This is the first
      report of the immortalization of MCECs. Ion transport of the immortalized
      endothelial cells remains active, except for NH3:H+ transporter activity in
      Slc4a11-/- MCECs. Furthermore, Slc4a11-/- MCECs recapitulate the glutaminolysis
      defects observed in Slc4a11-/- mouse corneal endothelium, providing an excellent 
      tool to study the pathogenesis of SLC4A11 mutations associated with corneal
      endothelial dystrophies and to screen potential therapeutic agents.
FAU - Zhang, Wenlin
AU  - Zhang W
AD  - School of Optometry, Indiana University, Bloomington, Indiana, United States.
FAU - Ogando, Diego G
AU  - Ogando DG
AD  - School of Optometry, Indiana University, Bloomington, Indiana, United States.
FAU - Kim, Edward T
AU  - Kim ET
AD  - School of Optometry, Indiana University, Bloomington, Indiana, United States.
FAU - Choi, Moon-Jung
AU  - Choi MJ
AD  - School of Optometry, Indiana University, Bloomington, Indiana, United States.
FAU - Li, Hongde
AU  - Li H
AD  - Department of Biology, Indiana University, Bloomington, Indiana, United States.
FAU - Tenessen, Jason M
AU  - Tenessen JM
AD  - Department of Biology, Indiana University, Bloomington, Indiana, United States.
FAU - Bonanno, Joseph A
AU  - Bonanno JA
AD  - School of Optometry, Indiana University, Bloomington, Indiana, United States.
LA  - eng
GR  - P30 EY019008/EY/NEI NIH HHS/United States
GR  - R00 GM101341/GM/NIGMS NIH HHS/United States
GR  - R01 EY008834/EY/NEI NIH HHS/United States
GR  - R35 GM119557/GM/NIGMS NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
RN  - 0 (Anion Transport Proteins)
RN  - 0 (Antigens, Polyomavirus Transforming)
RN  - 0 (Slc4a11 protein, mouse)
RN  - 0 (Symporters)
RN  - 0RH81L854J (Glutamine)
SB  - IM
MH  - Animals
MH  - Anion Transport Proteins/*genetics/metabolism
MH  - Antigens, Polyomavirus Transforming/genetics
MH  - Blotting, Western
MH  - Cell Line
MH  - Cell Proliferation/physiology
MH  - Corneal Dystrophies, Hereditary/genetics/*metabolism/pathology
MH  - Disease Models, Animal
MH  - Endothelium, Corneal/*metabolism/pathology
MH  - Female
MH  - Gas Chromatography-Mass Spectrometry
MH  - Glutamine/*metabolism
MH  - Ion Transport/physiology
MH  - Male
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Knockout
MH  - Mice, Transgenic
MH  - Real-Time Polymerase Chain Reaction
MH  - Symporters/*genetics
PMC - PMC5525555
EDAT- 2017/07/25 06:00
MHDA- 2017/07/29 06:00
CRDT- 2017/07/25 06:00
AID - 2645902 [pii]
AID - 10.1167/iovs.17-21781 [doi]
PST - ppublish
SO  - Invest Ophthalmol Vis Sci. 2017 Jul 1;58(9):3723-3731. doi:
      10.1167/iovs.17-21781.