NCBI Home Page NCBI Site Search page NCBI Guide that lists and describes the NCBI resources
Conserved domains on  [gi|1755203708|ref|NP_001361423|]
View 

cystinosin isoform 3 [Homo sapiens]

Protein Classification

cystinosin/MPDU1 family protein( domain architecture ID 11490013)

cystinosin/MPDU1 family protein similar to homo sapiens mannose-P-dolichol utilization defect 1, a cystinosin homolog that required for normal utilization of mannose-dolichol phosphate (Dol-P-Man) in the synthesis of N-linked and O-linked oligosaccharides and GPI anchors

Graphical summary

 Zoom to residue level

show extra options »

Show site features     Horizontal zoom: ×

List of domain hits

 Name Accession Description Interval E-value
2A43 TIGR00951
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ...
 1-210 1.75e-81

Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines]


:

Pssm-ID: 130026 [Multi-domain]  Cd Length: 220  Bit Score: 242.07  E-value: 1.75e-81
                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708   1 MNWRRKSVIGLSFDFVALNLTGFVAYSVFNIGLLWVPYIKEQFLLKYPNgvnpVNSNDVFFSLHAVVLTLIIIVQCCLYE 80
Cdd:TIGR00951  25 KNWRRKSAEGLSFDFVMLNLVGFTAYVIFNFLQLYCWSITNEFPLSSPG----VTQNDVFFTLHAILICFIVLHQCGDYE 100

                          90       100       110       120       130       140       150       160
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708  81 RGGQRVSWPAIGFLVLAWLFAFVTMIVAAVGVTTWLQFLFCFSYIKLAVTLVKYFPQAYMNFYYKSTEGWSIGNVLLDFT 160
Cdd:TIGR00951 101 RGWQRVSNPWILRILVALLACFATLLVALLSPITPLAFVTMLSYIKVAVTLVKYFPQAATNYHNKSTGQLSIITVFLDFT 180

                         170       180       190       200       210
                  ....*....|....*....|....*....|....*....|....*....|
gi 1755203708 161 GgsfsLLQMFLQSYNNDqwtlifGDPTKFGLGVFSIVFDVVFFIQHFCLY 210
Cdd:TIGR00951 181 G----LLQRIFQSVNET------GDPLKAGLFVVSSLFNGLFAAQVFFYW 220
 
Name Accession Description Interval E-value
2A43 TIGR00951
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ...
 1-210 1.75e-81

Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines]


Pssm-ID: 130026 [Multi-domain]  Cd Length: 220  Bit Score: 242.07  E-value: 1.75e-81
                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708   1 MNWRRKSVIGLSFDFVALNLTGFVAYSVFNIGLLWVPYIKEQFLLKYPNgvnpVNSNDVFFSLHAVVLTLIIIVQCCLYE 80
Cdd:TIGR00951  25 KNWRRKSAEGLSFDFVMLNLVGFTAYVIFNFLQLYCWSITNEFPLSSPG----VTQNDVFFTLHAILICFIVLHQCGDYE 100

                          90       100       110       120       130       140       150       160
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708  81 RGGQRVSWPAIGFLVLAWLFAFVTMIVAAVGVTTWLQFLFCFSYIKLAVTLVKYFPQAYMNFYYKSTEGWSIGNVLLDFT 160
Cdd:TIGR00951 101 RGWQRVSNPWILRILVALLACFATLLVALLSPITPLAFVTMLSYIKVAVTLVKYFPQAATNYHNKSTGQLSIITVFLDFT 180

                         170       180       190       200       210
                  ....*....|....*....|....*....|....*....|....*....|
gi 1755203708 161 GgsfsLLQMFLQSYNNDqwtlifGDPTKFGLGVFSIVFDVVFFIQHFCLY 210
Cdd:TIGR00951 181 G----LLQRIFQSVNET------GDPLKAGLFVVSSLFNGLFAAQVFFYW 220
PQ-loop pfam04193
PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ...
 118-178 3.22e-12

PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of repeats each spanning two transmembrane helices connected by a loop. The PQ motif found on loop 2 is critical for the localization of cystinosin to lysosomes. However, the PQ motif appears not to be a general lysosome-targeting motif. It is thought likely to possess a more general function. Most probably this involves a glutamine residue. Family members are membrane transporters since two members, cystinosin and PQLC2, transport cystine and cationic amino acids, respectively, across the lysosomal membrane. The 2nd PQ-loop of cystinosin hosts the substrate-coupled H+ binding site underlying its H+ symport mechanism, suggesting that PQ-loop repeats have functional significance. It is thus likely that PQ-loop-containing proteins act as a family of membrane transporters. Some transport cystine and cationic amino acids, respectively, across the lysosomal membrane. Others transport lysine and or arginine across the lysosomal membrane in order to maintain the acidic homoeostasis.


Pssm-ID: 461220 [Multi-domain]  Cd Length: 61  Bit Score: 59.44  E-value: 3.22e-12
                          10        20        30        40        50        60
                  ....*....|....*....|....*....|....*....|....*....|....*....|.
gi 1755203708 118 FLFCFSYIKLAVTLVKYFPQAYMNFYYKSTEGWSIGNVLLDFTGGSFSLLQMFLQSYNNDQ 178
Cdd:pfam04193   1 LSNVLGYISLVLWLVSYLPQIIKNYKRKSTEGLSILFLLLWLLGDIFYLLYLILNGFPPSL 61
CTNS smart00679
Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ...
 132-163 1.64e-04

Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, mannose-P-dolichol utilization defect 1, and other hypothetical proteins; Function unknown, but likely to be associated with the glycosylation machinery.


Pssm-ID: 128923 [Multi-domain]  Cd Length: 32  Bit Score: 37.82  E-value: 1.64e-04
                           10        20        30
                   ....*....|....*....|....*....|..
gi 1755203708  132 VKYFPQAYMNFYYKSTEGWSIGNVLLDFTGGS 163
Cdd:smart00679   1 VSLLPQIIKNYRRKSTEGLSILFVLLWLLGDI 32
 
Name Accession Description Interval E-value
2A43 TIGR00951
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ...
 1-210 1.75e-81

Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines]


Pssm-ID: 130026 [Multi-domain]  Cd Length: 220  Bit Score: 242.07  E-value: 1.75e-81
                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708   1 MNWRRKSVIGLSFDFVALNLTGFVAYSVFNIGLLWVPYIKEQFLLKYPNgvnpVNSNDVFFSLHAVVLTLIIIVQCCLYE 80
Cdd:TIGR00951  25 KNWRRKSAEGLSFDFVMLNLVGFTAYVIFNFLQLYCWSITNEFPLSSPG----VTQNDVFFTLHAILICFIVLHQCGDYE 100

                          90       100       110       120       130       140       150       160
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 1755203708  81 RGGQRVSWPAIGFLVLAWLFAFVTMIVAAVGVTTWLQFLFCFSYIKLAVTLVKYFPQAYMNFYYKSTEGWSIGNVLLDFT 160
Cdd:TIGR00951 101 RGWQRVSNPWILRILVALLACFATLLVALLSPITPLAFVTMLSYIKVAVTLVKYFPQAATNYHNKSTGQLSIITVFLDFT 180

                         170       180       190       200       210
                  ....*....|....*....|....*....|....*....|....*....|
gi 1755203708 161 GgsfsLLQMFLQSYNNDqwtlifGDPTKFGLGVFSIVFDVVFFIQHFCLY 210
Cdd:TIGR00951 181 G----LLQRIFQSVNET------GDPLKAGLFVVSSLFNGLFAAQVFFYW 220
PQ-loop pfam04193
PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ...
 118-178 3.22e-12

PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of repeats each spanning two transmembrane helices connected by a loop. The PQ motif found on loop 2 is critical for the localization of cystinosin to lysosomes. However, the PQ motif appears not to be a general lysosome-targeting motif. It is thought likely to possess a more general function. Most probably this involves a glutamine residue. Family members are membrane transporters since two members, cystinosin and PQLC2, transport cystine and cationic amino acids, respectively, across the lysosomal membrane. The 2nd PQ-loop of cystinosin hosts the substrate-coupled H+ binding site underlying its H+ symport mechanism, suggesting that PQ-loop repeats have functional significance. It is thus likely that PQ-loop-containing proteins act as a family of membrane transporters. Some transport cystine and cationic amino acids, respectively, across the lysosomal membrane. Others transport lysine and or arginine across the lysosomal membrane in order to maintain the acidic homoeostasis.


Pssm-ID: 461220 [Multi-domain]  Cd Length: 61  Bit Score: 59.44  E-value: 3.22e-12
                          10        20        30        40        50        60
                  ....*....|....*....|....*....|....*....|....*....|....*....|.
gi 1755203708 118 FLFCFSYIKLAVTLVKYFPQAYMNFYYKSTEGWSIGNVLLDFTGGSFSLLQMFLQSYNNDQ 178
Cdd:pfam04193   1 LSNVLGYISLVLWLVSYLPQIIKNYKRKSTEGLSILFLLLWLLGDIFYLLYLILNGFPPSL 61
PQ-loop pfam04193
PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ...
 1-39 1.76e-05

PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of repeats each spanning two transmembrane helices connected by a loop. The PQ motif found on loop 2 is critical for the localization of cystinosin to lysosomes. However, the PQ motif appears not to be a general lysosome-targeting motif. It is thought likely to possess a more general function. Most probably this involves a glutamine residue. Family members are membrane transporters since two members, cystinosin and PQLC2, transport cystine and cationic amino acids, respectively, across the lysosomal membrane. The 2nd PQ-loop of cystinosin hosts the substrate-coupled H+ binding site underlying its H+ symport mechanism, suggesting that PQ-loop repeats have functional significance. It is thus likely that PQ-loop-containing proteins act as a family of membrane transporters. Some transport cystine and cationic amino acids, respectively, across the lysosomal membrane. Others transport lysine and or arginine across the lysosomal membrane in order to maintain the acidic homoeostasis.


Pssm-ID: 461220 [Multi-domain]  Cd Length: 61  Bit Score: 41.33  E-value: 1.76e-05
                          10        20        30
                  ....*....|....*....|....*....|....*....
gi 1755203708   1 MNWRRKSVIGLSFDFVALNLTGFVAYSVFNIGLLWVPYI 39
Cdd:pfam04193  23 KNYKRKSTEGLSILFLLLWLLGDIFYLLYLILNGFPPSL 61
CTNS smart00679
Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ...
 132-163 1.64e-04

Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, mannose-P-dolichol utilization defect 1, and other hypothetical proteins; Function unknown, but likely to be associated with the glycosylation machinery.


Pssm-ID: 128923 [Multi-domain]  Cd Length: 32  Bit Score: 37.82  E-value: 1.64e-04
                           10        20        30
                   ....*....|....*....|....*....|..
gi 1755203708  132 VKYFPQAYMNFYYKSTEGWSIGNVLLDFTGGS 163
Cdd:smart00679   1 VSLLPQIIKNYRRKSTEGLSILFVLLWLLGDI 32
CTNS smart00679
Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ...
 1-24 2.58e-03

Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, mannose-P-dolichol utilization defect 1, and other hypothetical proteins; Function unknown, but likely to be associated with the glycosylation machinery.


Pssm-ID: 128923 [Multi-domain]  Cd Length: 32  Bit Score: 34.35  E-value: 2.58e-03
                           10        20
                   ....*....|....*....|....
gi 1755203708    1 MNWRRKSVIGLSFDFVALNLTGFV 24
Cdd:smart00679   9 KNYRRKSTEGLSILFVLLWLLGDI 32
 
Blast search parameters
Data Source: Precalculated data, version = cdd.v.3.21
Preset Options:Database: CDSEARCH/cdd   Low complexity filter: no  Composition Based Adjustment: yes   E-value threshold: 0.01

References:

  • Wang J et al. (2023), "The conserved domain database in 2023", Nucleic Acids Res.51(D)384-8.
  • Lu S et al. (2020), "The conserved domain database in 2020", Nucleic Acids Res.48(D)265-8.
  • Marchler-Bauer A et al. (2017), "CDD/SPARCLE: functional classification of proteins via subfamily domain architectures.", Nucleic Acids Res.45(D)200-3.
Help | Disclaimer | Write to the Help Desk
NCBI | NLM | NIH