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2.A.22.6.3
Kidney and intestinal apical membrane epithelial transporter for Na+-dependent, Cl--independent reabsorption of neutral amino acids. Many neutral L-amino acids bind with ~0.5 mM affinities Leu is the preferred substrate, but all large neutral non-aromatic L-amino acids bind to this transporter. Uptake of leucine is sodium-dependent. In contrast to other members of the neurotransmitter transporter family, this one does not appear to be chloride-dependent.  Activity is enhanced by collectrin (Tmem27), a collecting duct transmembrane (1 TMS) glycoprotein (Q9HBJ8) (Danilczyk et al., 2006). The Hartnup Disorder protein (mouse orthologue, (Q9D687) (Broer et al., 2004; 2008) forms a complex with collectrin and the brush border carboxypeptidase angiotensin-converting enzyme 2 (ACE2). Mutation as in Hartnup disorder (B0AT1(R240Q)) decreases complex formation and leads to neutral aminoaciduria and in some cases pellagra-like symptoms (Kowalczuk et al., 2008; Singer et al. 2012).  Collectrin is expressed in the simple embryonic kidney of amphibians such as Xenopus, the pronephros, at high levels (McCoy et al. 2008).           

Accession Number:Q9D687
Protein Name:Sodium-dependent neutral amino acid transporter B(0) aka B0at1
Length:634
Molecular Weight:71367.00
Species:Mus musculus (Mouse) [10090]
Number of TMSs:12
Location1 / Topology2 / Orientation3: Membrane1 / Multi-pass membrane protein2
Substrate Na+, Cl-, Neutral amino acids

Cross database links:

Genevestigator: Q9D687
HEGENOM: HBG702834
RefSeq: NP_083154.1   
Entrez Gene ID: 74338   
Pfam: PF00209   
KEGG: mmu:74338   

Gene Ontology

GO:0005887 C:integral to plasma membrane
GO:0005328 F:neurotransmitter:sodium symporter activity
GO:0015175 F:neutral amino acid transmembrane transporte...
GO:0006836 P:neurotransmitter transport
GO:0015804 P:neutral amino acid transport

References (3)

[1] “Molecular cloning of mouse amino acid transport system B(0), a neutral amino acid transporter related to Hartnup disorder.”  Broeer A.et.al.   15044460
[2] “The transcriptional landscape of the mammalian genome.”  Carninci P.et.al.   16141072
[3] “Characterization of mouse amino acid transporter B(0)AT1 (Slc6a19).”  Boehmer C.et.al.   15804236

External Searches:

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  • CDD Search (Conserved Domain Database)
  • Search COGs (Clusters of Orthologous Groups of proteins)
  • 2° Structure (Network Protein Sequence Analysis)

Analyze:

Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MVRLVLPNPG LEERIPSLDE LEVIEKEEAG SRPKWDNKAQ YMLTCVGFCV GLGNVWRFPY 
61:	LCQSHGGGAF MIPFLILLVF EGIPLLYLEF AIGQRLRKGS MGVWSSIHPA LKGIGIASMF 
121:	VSFMVGLYYN TIIAWVMWYF FNSFQEPLPW SECPLNQNQT GYVEECAKSS SVDYFWYRET 
181:	LNISTSISDS GSIQWWILLC LTCAWSVLYV CIIRGIETTG KAVYITSTLP YVVLTIFLIR 
241:	GLTLKGATNG IVFLFTPNIT ELSNPNTWLD AGAQVFYSFS LAFGGLISFS SYNSVHNNCE 
301:	MDSVIVSVIN GFTSVYAATV VYSIIGFRAT ERFDDCVNTN ILTLINGFDL PEGNVTSENF 
361:	EAYQQWCNAT NPQAYAQLKF QTCDINSFLS EGVEGTGLAF IVFTEAITKM PVSPLWSVLF 
421:	FIMLFCLGLS SMFGNMEGVV VPLQDLNITP KKWPKELLTG LICLGTYLIA FIFTLNSGQY 
481:	WLSLLDSFAG SIPLLIIAFC EMFAVVYVYG VDRFNKDIEF MIGHKPNIFW QVTWRVVSPL 
541:	IMLVIFLFFF VIEVNKTLMY SIWDPNYEEF PKSQKIPYPN WVYAVVVTVA GVPCLSIPCF 
601:	AIYKFIRNCC QKSDDHHGLV NTLSTASVNG DLKN