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Pathways
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PATHWAY ONTOLOGY - ANNOTATIONS

The Pathway Ontology (PW), is currently being developed at the Rat Genome Database. For more information about this vocabulary, please see Petri et al. The rat genome database pathway portal. Database (Oxford). 2011 Apr 8;2011:bar010. Print 2011 or contact us (http://rgd.mcw.edu/contact/index.shtml).
Term:lysinuric protein intolerance pathway
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Accession:PW:0001871 term browser browse the term
Definition:A disorder resulting from an impaired transport pathway of positively charged amino acids.
Synonyms:exact_synonym: LPI pathway;   dibasic amino aciduria II pathway;   hyperdibasic aminoaciduria pathway;   lysinuric protein intolerance disease pathway
 related_synonym: SMP:00197;   SMP:00585

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lysinuric protein intolerance pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Aqp1 aquaporin 1 (Colton blood group) ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936478:7,146,997...7,160,369
Ensembl chrNW_004936478:7,147,027...7,160,401 		JBrowse link
G Aqp2 aquaporin 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936512:7,628,677...7,633,344
Ensembl chrNW_004936512:7,628,495...7,634,107 		JBrowse link
G Aqp3 aquaporin 3 (Gill blood group) ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936524:1,963,427...1,969,977
Ensembl chrNW_004936524:1,963,247...1,969,998 		JBrowse link
G Atp1a1 ATPase Na+/K+ transporting subunit alpha 1 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936627:1,555,151...1,584,370
Ensembl chrNW_004936627:1,554,873...1,584,638 		JBrowse link
G Atp1a2 ATPase Na+/K+ transporting subunit alpha 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936740:489,357...514,235
Ensembl chrNW_004936740:488,154...514,308 		JBrowse link
G Atp1a3 ATPase Na+/K+ transporting subunit alpha 3 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936706:476,473...494,659
Ensembl chrNW_004936706:477,107...495,525 		JBrowse link
G Atp1b1 ATPase Na+/K+ transporting subunit beta 1 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936481:17,438,146...17,460,329
Ensembl chrNW_004936481:17,436,270...17,460,348 		JBrowse link
G Atp1b2 ATPase Na+/K+ transporting subunit beta 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936595:908,019...914,426
Ensembl chrNW_004936595:907,919...915,053 		JBrowse link
G Atp1b3 ATPase Na+/K+ transporting subunit beta 3 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936540:3,621,656...3,640,885 JBrowse link
G Atp1b4 ATPase Na+/K+ transporting family member beta 4 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936479:9,648,315...9,666,782
Ensembl chrNW_004936479:9,650,598...9,666,782 		JBrowse link
G Clcnka chloride voltage-gated channel Ka ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936474:3,591,134...3,601,562 JBrowse link
G Fxyd2 FXYD domain containing ion transport regulator 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936542:2,956,784...2,961,076
Ensembl chrNW_004936542:2,956,785...2,963,506 		JBrowse link
G LOC101965929 urea transporter 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936517:1,060,311...1,104,775
Ensembl chrNW_004936517:1,061,119...1,104,768 		JBrowse link
G LOC101966280 sodium/potassium-transporting ATPase subunit alpha-4 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936740:445,952...478,580
Ensembl chrNW_004936740:445,952...478,532 		JBrowse link
G Nr3c2 nuclear receptor subfamily 3 group C member 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936535:1,139,687...1,467,469
Ensembl chrNW_004936535:1,140,178...1,467,471 		JBrowse link
G Scnn1a sodium channel epithelial 1 subunit alpha ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936709:1,377,852...1,403,398
Ensembl chrNW_004936709:1,377,831...1,403,833 		JBrowse link
G Scnn1b sodium channel epithelial 1 subunit beta ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936501:8,021,090...8,065,245
Ensembl chrNW_004936501:8,020,316...8,065,332 		JBrowse link
G Scnn1d sodium channel epithelial 1 subunit delta ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936737:1,851,185...1,854,923 JBrowse link
G Scnn1g sodium channel epithelial 1 subunit gamma ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936501:7,932,729...7,958,146
Ensembl chrNW_004936501:7,932,703...7,958,204 		JBrowse link
G Slc12a1 solute carrier family 12 member 1 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936471:10,564,530...10,648,124
Ensembl chrNW_004936471:10,564,535...10,646,977 		JBrowse link
G Slc12a3 solute carrier family 12 member 3 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936475:9,044,099...9,076,301
Ensembl chrNW_004936475:9,044,106...9,076,307 		JBrowse link
G Slc12a6 solute carrier family 12 member 6 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936673:2,731,454...2,825,179
Ensembl chrNW_004936673:2,727,884...2,824,977 		JBrowse link
G Slc1a1 solute carrier family 1 member 1 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936503:613,250...690,555
Ensembl chrNW_004936503:613,223...690,721 		JBrowse link
G Slc22a2 solute carrier family 22 member 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936489:11,605,403...11,627,786
Ensembl chrNW_004936489:11,594,480...11,627,786 		JBrowse link
G Slc22a6 solute carrier family 22 member 6 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936581:348,719...356,246
Ensembl chrNW_004936581:349,057...356,059 		JBrowse link
G Slc38a4 solute carrier family 38 member 4 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936512:4,867,276...4,929,175
Ensembl chrNW_004936512:4,865,769...4,929,166 		JBrowse link
G Slc3a1 solute carrier family 3 member 1 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936508:7,672,274...7,703,962
Ensembl chrNW_004936508:7,672,223...7,703,961 		JBrowse link
G Slc3a2 solute carrier family 3 member 2 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936581:424,801...441,592 JBrowse link
G Slc6a20 solute carrier family 6 member 20 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936695:146,571...193,428
Ensembl chrNW_004936695:146,421...195,121 		JBrowse link
G Slc7a5 solute carrier family 7 member 5 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936641:1,513,110...1,538,955 JBrowse link
G Slc7a6 solute carrier family 7 member 6 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936475:18,587,058...18,624,757
Ensembl chrNW_004936475:18,586,931...18,625,137 		JBrowse link
G Slc7a7 solute carrier family 7 member 7 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936722:1,464,252...1,523,786
Ensembl chrNW_004936722:1,478,240...1,527,121 		JBrowse link
G Slc7a8 solute carrier family 7 member 8 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936722:1,143,405...1,202,407
Ensembl chrNW_004936722:1,143,309...1,202,514 		JBrowse link
G Slc7a9 solute carrier family 7 member 9 ISO SMPDB SMP:00197 SMP:00585 NCBI chrNW_004936570:2,917,389...2,944,452
Ensembl chrNW_004936570:2,917,709...2,944,158 		JBrowse link
Term paths to the root
Path 1
Term Annotations click to browse term
  pathway 3944
    disease pathway 688
      congenital disease pathway 507
        inborn genetic disease pathway 507
          inborn error of metabolism pathway 507
            inborn error of amino acid metabolism pathway 222
              lysinuric protein intolerance pathway 34
paths to the root