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Disorder of neutral amino acid transport

MedGen UID:
831653
Concept ID:
CN203583
Disease or Syndrome
Synonyms: disorder of neutral amino acid transport; inborn error of neutral amino acid transport; inborn neutral amino acid transport disorder; rare inborn error of neutral amino acid transport
 
Monarch Initiative: MONDO:0017687
Orphanet: ORPHA308451

Definition

An inherited metabolic disease that is has its basis in the disruption of neutral amino acid transport. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDisorder of neutral amino acid transport
Follow this link to review classifications for Disorder of neutral amino acid transport in Orphanet.

Professional guidelines

PubMed

Jamalpoor A, Othman A, Levtchenko EN, Masereeuw R, Janssen MJ
Trends Mol Med 2021 Jul;27(7):673-686. Epub 2021 May 8 doi: 10.1016/j.molmed.2021.04.004. PMID: 33975805
Daga S, Palit V, Forster JA, Biyani CS, Joyce AD, Dimitrova AB
Urology 2021 Mar;149:70-75. Epub 2021 Jan 6 doi: 10.1016/j.urology.2020.12.025. PMID: 33421442
Saravakos P, Kokkinou V, Giannatos E
Urology 2014 Apr;83(4):693-9. Epub 2013 Nov 16 doi: 10.1016/j.urology.2013.10.013. PMID: 24246330

Recent clinical studies

Etiology

Juliá-Palacios N, Olivella M, Sigatullina Bondarenko M, Ibáñez-Micó S, Muñoz-Cabello B, Alonso-Luengo O, Soto-Insuga V, García-Navas D, Cuesta-Herraiz L, Andreo-Lillo P, Aguilera-Albesa S, Hedrera-Fernández A, González Alguacil E, Sánchez-Carpintero R, Martín Del Valle F, Jiménez González E, Cean Cabrera L, Medina-Rivera I, Perez-Ordoñez M, Colomé R, Lopez L, Engracia Cazorla M, Fornaguera M, Ormazabal A, Alonso-Colmenero I, Illescas KS, Balsells-Mejía S, Mari-Vico R, Duffo Viñas M, Cappuccio G, Terrone G, Romano R, Manti F, Mastrangelo M, Alfonsi C, de Siqueira Barros B, Nizon M, Gjerulfsen CE, Muro VL, Karall D, Zeiner F, Masnada S, Peterlongo I, Oyarzábal A, Santos-Gómez A, Altafaj X, García-Cazorla Á
Brain 2024 May 3;147(5):1653-1666. doi: 10.1093/brain/awae041. PMID: 38380699
Cheon H, Xing JC, Moosic KB, Ung J, Chan VW, Chung DS, Toro MF, Elghawy O, Wang JS, Hamele CE, Hardison RC, Olson TL, Tan SF, Feith DJ, Ratan A, Loughran TP
Blood 2022 May 19;139(20):3058-3072. doi: 10.1182/blood.2021013164. PMID: 35015834Free PMC Article
van Rheenen W, van der Spek RAA, Bakker MK, van Vugt JJFA, Hop PJ, Zwamborn RAJ, de Klein N, Westra HJ, Bakker OB, Deelen P, Shireby G, Hannon E, Moisse M, Baird D, Restuadi R, Dolzhenko E, Dekker AM, Gawor K, Westeneng HJ, Tazelaar GHP, van Eijk KR, Kooyman M, Byrne RP, Doherty M, Heverin M, Al Khleifat A, Iacoangeli A, Shatunov A, Ticozzi N, Cooper-Knock J, Smith BN, Gromicho M, Chandran S, Pal S, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Sendtner M, Meyer T, Başak N, van der Kooi AJ, Ratti A, Fogh I, Gellera C, Lauria G, Corti S, Cereda C, Sproviero D, D'Alfonso S, Sorarù G, Siciliano G, Filosto M, Padovani A, Chiò A, Calvo A, Moglia C, Brunetti M, Canosa A, Grassano M, Beghi E, Pupillo E, Logroscino G, Nefussy B, Osmanovic A, Nordin A, Lerner Y, Zabari M, Gotkine M, Baloh RH, Bell S, Vourc'h P, Corcia P, Couratier P, Millecamps S, Meininger V, Salachas F, Mora Pardina JS, Assialioui A, Rojas-García R, Dion PA, Ross JP, Ludolph AC, Weishaupt JH, Brenner D, Freischmidt A, Bensimon G, Brice A, Durr A, Payan CAM, Saker-Delye S, Wood NW, Topp S, Rademakers R, Tittmann L, Lieb W, Franke A, Ripke S, Braun A, Kraft J, Whiteman DC, Olsen CM, Uitterlinden AG, Hofman A, Rietschel M, Cichon S, Nöthen MM, Amouyel P; SLALOM Consortium; PARALS Consortium; SLAGEN Consortium; SLAP Consortium, Traynor BJ, Singleton AB, Mitne Neto M, Cauchi RJ, Ophoff RA, Wiedau-Pazos M, Lomen-Hoerth C, van Deerlin VM, Grosskreutz J, Roediger A, Gaur N, Jörk A, Barthel T, Theele E, Ilse B, Stubendorff B, Witte OW, Steinbach R, Hübner CA, Graff C, Brylev L, Fominykh V, Demeshonok V, Ataulina A, Rogelj B, Koritnik B, Zidar J, Ravnik-Glavač M, Glavač D, Stević Z, Drory V, Povedano M, Blair IP, Kiernan MC, Benyamin B, Henderson RD, Furlong S, Mathers S, McCombe PA, Needham M, Ngo ST, Nicholson GA, Pamphlett R, Rowe DB, Steyn FJ, Williams KL, Mather KA, Sachdev PS, Henders AK, Wallace L, de Carvalho M, Pinto S, Petri S, Weber M, Rouleau GA, Silani V, Curtis CJ, Breen G, Glass JD, Brown RH Jr, Landers JE, Shaw CE, Andersen PM, Groen EJN, van Es MA, Pasterkamp RJ, Fan D, Garton FC, McRae AF, Davey Smith G, Gaunt TR, Eberle MA, Mill J, McLaughlin RL, Hardiman O, Kenna KP, Wray NR, Tsai E, Runz H, Franke L, Al-Chalabi A, Van Damme P, van den Berg LH, Veldink JH
Nat Genet 2021 Dec;53(12):1636-1648. Epub 2021 Dec 6 doi: 10.1038/s41588-021-00973-1. PMID: 34873335Free PMC Article
Nikolaus S, Schulte B, Al-Massad N, Thieme F, Schulte DM, Bethge J, Rehman A, Tran F, Aden K, Häsler R, Moll N, Schütze G, Schwarz MJ, Waetzig GH, Rosenstiel P, Krawczak M, Szymczak S, Schreiber S
Gastroenterology 2017 Dec;153(6):1504-1516.e2. Epub 2017 Aug 19 doi: 10.1053/j.gastro.2017.08.028. PMID: 28827067
Tărlungeanu DC, Deliu E, Dotter CP, Kara M, Janiesch PC, Scalise M, Galluccio M, Tesulov M, Morelli E, Sonmez FM, Bilguvar K, Ohgaki R, Kanai Y, Johansen A, Esharif S, Ben-Omran T, Topcu M, Schlessinger A, Indiveri C, Duncan KE, Caglayan AO, Gunel M, Gleeson JG, Novarino G
Cell 2016 Dec 1;167(6):1481-1494.e18. doi: 10.1016/j.cell.2016.11.013. PMID: 27912058Free PMC Article

Diagnosis

Wang Z, Ouyang L, Liu N, Li T, Yan B, Mao C, Xiao D, Gan B, Liu S, Tao Y
Oncogene 2023 Sep;42(36):2688-2700. Epub 2023 Aug 3 doi: 10.1038/s41388-023-02744-0. PMID: 37537342
Elhawary NA, AlJahdali IA, Abumansour IS, Elhawary EN, Gaboon N, Dandini M, Madkhali A, Alosaimi W, Alzahrani A, Aljohani F, Melibary EM, Kensara OA
Hum Genomics 2022 Jul 19;16(1):22. doi: 10.1186/s40246-022-00398-9. PMID: 35854334Free PMC Article
Amaya ML, Inguva A, Pei S, Jones C, Krug A, Ye H, Minhajuddin M, Winters A, Furtek SL, Gamboni F, Stevens B, D'Alessandro A, Pollyea DA, Reigan P, Jordan CT
Blood 2022 Jan 27;139(4):584-596. doi: 10.1182/blood.2021013201. PMID: 34525179Free PMC Article
Nikolaus S, Schulte B, Al-Massad N, Thieme F, Schulte DM, Bethge J, Rehman A, Tran F, Aden K, Häsler R, Moll N, Schütze G, Schwarz MJ, Waetzig GH, Rosenstiel P, Krawczak M, Szymczak S, Schreiber S
Gastroenterology 2017 Dec;153(6):1504-1516.e2. Epub 2017 Aug 19 doi: 10.1053/j.gastro.2017.08.028. PMID: 28827067
Elmonem MA, Veys KR, Soliman NA, van Dyck M, van den Heuvel LP, Levtchenko E
Orphanet J Rare Dis 2016 Apr 22;11:47. doi: 10.1186/s13023-016-0426-y. PMID: 27102039Free PMC Article

Therapy

Elhawary NA, AlJahdali IA, Abumansour IS, Elhawary EN, Gaboon N, Dandini M, Madkhali A, Alosaimi W, Alzahrani A, Aljohani F, Melibary EM, Kensara OA
Hum Genomics 2022 Jul 19;16(1):22. doi: 10.1186/s40246-022-00398-9. PMID: 35854334Free PMC Article
van Rheenen W, van der Spek RAA, Bakker MK, van Vugt JJFA, Hop PJ, Zwamborn RAJ, de Klein N, Westra HJ, Bakker OB, Deelen P, Shireby G, Hannon E, Moisse M, Baird D, Restuadi R, Dolzhenko E, Dekker AM, Gawor K, Westeneng HJ, Tazelaar GHP, van Eijk KR, Kooyman M, Byrne RP, Doherty M, Heverin M, Al Khleifat A, Iacoangeli A, Shatunov A, Ticozzi N, Cooper-Knock J, Smith BN, Gromicho M, Chandran S, Pal S, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Sendtner M, Meyer T, Başak N, van der Kooi AJ, Ratti A, Fogh I, Gellera C, Lauria G, Corti S, Cereda C, Sproviero D, D'Alfonso S, Sorarù G, Siciliano G, Filosto M, Padovani A, Chiò A, Calvo A, Moglia C, Brunetti M, Canosa A, Grassano M, Beghi E, Pupillo E, Logroscino G, Nefussy B, Osmanovic A, Nordin A, Lerner Y, Zabari M, Gotkine M, Baloh RH, Bell S, Vourc'h P, Corcia P, Couratier P, Millecamps S, Meininger V, Salachas F, Mora Pardina JS, Assialioui A, Rojas-García R, Dion PA, Ross JP, Ludolph AC, Weishaupt JH, Brenner D, Freischmidt A, Bensimon G, Brice A, Durr A, Payan CAM, Saker-Delye S, Wood NW, Topp S, Rademakers R, Tittmann L, Lieb W, Franke A, Ripke S, Braun A, Kraft J, Whiteman DC, Olsen CM, Uitterlinden AG, Hofman A, Rietschel M, Cichon S, Nöthen MM, Amouyel P; SLALOM Consortium; PARALS Consortium; SLAGEN Consortium; SLAP Consortium, Traynor BJ, Singleton AB, Mitne Neto M, Cauchi RJ, Ophoff RA, Wiedau-Pazos M, Lomen-Hoerth C, van Deerlin VM, Grosskreutz J, Roediger A, Gaur N, Jörk A, Barthel T, Theele E, Ilse B, Stubendorff B, Witte OW, Steinbach R, Hübner CA, Graff C, Brylev L, Fominykh V, Demeshonok V, Ataulina A, Rogelj B, Koritnik B, Zidar J, Ravnik-Glavač M, Glavač D, Stević Z, Drory V, Povedano M, Blair IP, Kiernan MC, Benyamin B, Henderson RD, Furlong S, Mathers S, McCombe PA, Needham M, Ngo ST, Nicholson GA, Pamphlett R, Rowe DB, Steyn FJ, Williams KL, Mather KA, Sachdev PS, Henders AK, Wallace L, de Carvalho M, Pinto S, Petri S, Weber M, Rouleau GA, Silani V, Curtis CJ, Breen G, Glass JD, Brown RH Jr, Landers JE, Shaw CE, Andersen PM, Groen EJN, van Es MA, Pasterkamp RJ, Fan D, Garton FC, McRae AF, Davey Smith G, Gaunt TR, Eberle MA, Mill J, McLaughlin RL, Hardiman O, Kenna KP, Wray NR, Tsai E, Runz H, Franke L, Al-Chalabi A, Van Damme P, van den Berg LH, Veldink JH
Nat Genet 2021 Dec;53(12):1636-1648. Epub 2021 Dec 6 doi: 10.1038/s41588-021-00973-1. PMID: 34873335Free PMC Article
Zhang Z, Xu X, Tian W, Jiang R, Lu Y, Sun Q, Fu R, He Q, Wang J, Liu Y, Yu H, Sun B
J Hepatol 2020 May;72(5):976-989. Epub 2019 Dec 16 doi: 10.1016/j.jhep.2019.12.004. PMID: 31857195
Tărlungeanu DC, Deliu E, Dotter CP, Kara M, Janiesch PC, Scalise M, Galluccio M, Tesulov M, Morelli E, Sonmez FM, Bilguvar K, Ohgaki R, Kanai Y, Johansen A, Esharif S, Ben-Omran T, Topcu M, Schlessinger A, Indiveri C, Duncan KE, Caglayan AO, Gunel M, Gleeson JG, Novarino G
Cell 2016 Dec 1;167(6):1481-1494.e18. doi: 10.1016/j.cell.2016.11.013. PMID: 27912058Free PMC Article
Contin M, Martinelli P
J Neurol 2010 Nov;257(Suppl 2):S253-61. doi: 10.1007/s00415-010-5728-8. PMID: 21080186

Prognosis

Wong CC, Xu J, Bian X, Wu JL, Kang W, Qian Y, Li W, Chen H, Gou H, Liu D, Yat Luk ST, Zhou Q, Ji F, Chan LS, Shirasawa S, Sung JJ, Yu J
Gastroenterology 2020 Dec;159(6):2163-2180.e6. Epub 2020 Aug 16 doi: 10.1053/j.gastro.2020.08.016. PMID: 32814111
Le Douce J, Maugard M, Veran J, Matos M, Jégo P, Vigneron PA, Faivre E, Toussay X, Vandenberghe M, Balbastre Y, Piquet J, Guiot E, Tran NT, Taverna M, Marinesco S, Koyanagi A, Furuya S, Gaudin-Guérif M, Goutal S, Ghettas A, Pruvost A, Bemelmans AP, Gaillard MC, Cambon K, Stimmer L, Sazdovitch V, Duyckaerts C, Knott G, Hérard AS, Delzescaux T, Hantraye P, Brouillet E, Cauli B, Oliet SHR, Panatier A, Bonvento G
Cell Metab 2020 Mar 3;31(3):503-517.e8. doi: 10.1016/j.cmet.2020.02.004. PMID: 32130882
Song M, Sandoval TA, Chae CS, Chopra S, Tan C, Rutkowski MR, Raundhal M, Chaurio RA, Payne KK, Konrad C, Bettigole SE, Shin HR, Crowley MJP, Cerliani JP, Kossenkov AV, Motorykin I, Zhang S, Manfredi G, Zamarin D, Holcomb K, Rodriguez PC, Rabinovich GA, Conejo-Garcia JR, Glimcher LH, Cubillos-Ruiz JR
Nature 2018 Oct;562(7727):423-428. Epub 2018 Oct 10 doi: 10.1038/s41586-018-0597-x. PMID: 30305738Free PMC Article
Nikolaus S, Schulte B, Al-Massad N, Thieme F, Schulte DM, Bethge J, Rehman A, Tran F, Aden K, Häsler R, Moll N, Schütze G, Schwarz MJ, Waetzig GH, Rosenstiel P, Krawczak M, Szymczak S, Schreiber S
Gastroenterology 2017 Dec;153(6):1504-1516.e2. Epub 2017 Aug 19 doi: 10.1053/j.gastro.2017.08.028. PMID: 28827067
Liang J, Cao R, Wang X, Zhang Y, Wang P, Gao H, Li C, Yang F, Zeng R, Wei P, Li D, Li W, Yang W
Cell Res 2017 Mar;27(3):329-351. Epub 2016 Dec 30 doi: 10.1038/cr.2016.159. PMID: 28035139Free PMC Article

Clinical prediction guides

Cheon H, Xing JC, Moosic KB, Ung J, Chan VW, Chung DS, Toro MF, Elghawy O, Wang JS, Hamele CE, Hardison RC, Olson TL, Tan SF, Feith DJ, Ratan A, Loughran TP
Blood 2022 May 19;139(20):3058-3072. doi: 10.1182/blood.2021013164. PMID: 35015834Free PMC Article
Zhang Z, Xu X, Tian W, Jiang R, Lu Y, Sun Q, Fu R, He Q, Wang J, Liu Y, Yu H, Sun B
J Hepatol 2020 May;72(5):976-989. Epub 2019 Dec 16 doi: 10.1016/j.jhep.2019.12.004. PMID: 31857195
Yan R, Zhao X, Lei J, Zhou Q
Nature 2019 Apr;568(7750):127-130. Epub 2019 Mar 13 doi: 10.1038/s41586-019-1011-z. PMID: 30867591
Nikolaus S, Schulte B, Al-Massad N, Thieme F, Schulte DM, Bethge J, Rehman A, Tran F, Aden K, Häsler R, Moll N, Schütze G, Schwarz MJ, Waetzig GH, Rosenstiel P, Krawczak M, Szymczak S, Schreiber S
Gastroenterology 2017 Dec;153(6):1504-1516.e2. Epub 2017 Aug 19 doi: 10.1053/j.gastro.2017.08.028. PMID: 28827067
Contin M, Martinelli P
J Neurol 2010 Nov;257(Suppl 2):S253-61. doi: 10.1007/s00415-010-5728-8. PMID: 21080186

Recent systematic reviews

Soares C, Da Ros LU, Machado LS, Rocha A, Lazzarotto G, Carello-Collar G, De Bastiani MA, Ferrari-Souza JP, Lussier FZ, Souza DO, Rosa-Neto P, Pascoal TA, Bellaver B, Zimmer ER
Mol Psychiatry 2024 Jul;29(7):2261-2273. Epub 2024 Feb 16 doi: 10.1038/s41380-024-02473-0. PMID: 38366114
Lu JJ, Li P, Yang Y, Wang L, Zhang Y, Zhu JY, Zhu XR, Chen MB
PLoS One 2020;15(5):e0233629. Epub 2020 May 29 doi: 10.1371/journal.pone.0233629. PMID: 32469987Free PMC Article
Schade S, Paulus W
Int J Neuropsychopharmacol 2016 Apr;19(4) Epub 2016 Apr 20 doi: 10.1093/ijnp/pyv102. PMID: 26364274Free PMC Article

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