Cystinosis, nephropathic [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
List of domain hits
| Name | Accession | Description | Interval | E-value | ||||
| 2A43 | TIGR00951 | Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ... |
125-357 | 3.80e-99 | ||||
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines] : Pssm-ID: 130026 [Multi-domain] Cd Length: 220 Bit Score: 294.07 E-value: 3.80e-99
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| Name | Accession | Description | Interval | E-value | ||||
| 2A43 | TIGR00951 | Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ... |
125-357 | 3.80e-99 | ||||
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines] Pssm-ID: 130026 [Multi-domain] Cd Length: 220 Bit Score: 294.07 E-value: 3.80e-99
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| PQ-loop | pfam04193 | PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ... |
126-186 | 8.65e-16 | ||||
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: 70.99 E-value: 8.65e-16
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| CTNS | smart00679 | Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ... |
140-171 | 1.44e-07 | ||||
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: 47.06 E-value: 1.44e-07
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| Name | Accession | Description | Interval | E-value | ||||
| 2A43 | TIGR00951 | Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and ... |
125-357 | 3.80e-99 | ||||
Lysosomal Cystine Transporter; [Transport and binding proteins, Amino acids, peptides and amines] Pssm-ID: 130026 [Multi-domain] Cd Length: 220 Bit Score: 294.07 E-value: 3.80e-99
|
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| PQ-loop | pfam04193 | PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ... |
126-186 | 8.65e-16 | ||||
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: 70.99 E-value: 8.65e-16
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| PQ-loop | pfam04193 | PQ loop repeat; Members of this family are all membrane bound proteins possessing a pair of ... |
265-325 | 1.68e-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: 61.75 E-value: 1.68e-12
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| CTNS | smart00679 | Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ... |
140-171 | 1.44e-07 | ||||
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: 47.06 E-value: 1.44e-07
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| CTNS | smart00679 | Repeated motif present between transmembrane helices in cystinosin, yeast ERS1p, ... |
279-310 | 2.21e-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: 38.20 E-value: 2.21e-04
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| Blast search parameters | ||||
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