MFSD8 Polyclonal Antibody
Ucallm_WP2858-50
Price range: $268.00 through $328.00
Product details
Background:
This gene encodes a ubiquitous integral membrane protein that contains a transporter domain and a major facilitator superfamily (MFS) domain. Other members of the major facilitator superfamily transport small solutes through chemiosmotic ion gradients. The substrate transported by this protein is unknown. The protein likely localizes to lysosomal membranes. Mutations in this gene are correlated with a variant form of late infantile-onset neuronal ceroid lipofuscinoses (vLINCL). [provided by RefSeq, Oct 2008],
Specifications
| Size | 50μL/100μL |
| Target | MFSD8 |
| Reactivity | Human, Mouse |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 56kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000; ELISA 1:5000-20000 |
| Formulation | Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 351-400 |
| Specificity | MFSD8 Polyclonal Antibody detects endogenous levels of protein. |
| Gene Name | MFSD8 CLN7 |
| Protein Name | Major facilitator superfamily domain-containing protein 8 (Ceroid-lipofuscinosis neuronal protein 7) |
| Other Name | |
| Species | Human |
| Gene ID | 256471 |
| Uniprot | Q8NHS3, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q8BH31 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Lysosome membrane; Multi-pass membrane protein . Sorting to lysosomes involves tyrosine- and/or dileucine-based motifs. |
| Tissue Expression | Expressed at very low levels in all tissues tested. |
| Signaling_pathway | Cellular Processes >> Transport and catabolism >> Lysosome |
| Research Areas | >>Lysosome |
| Function | Disease:Defects in MFSD8 are the cause of neuronal ceroid lipofuscinosis type 7 (CLN7) [MIM:610951]. CNL are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The patterns most often observed CLN7 are mixed combinations of granular, curvilinear, fingerprint, and rectilinear profiles. The clinical course includes progressive dementia, seizures, and progressive visual failure.,Function:May be a carrier that transport small solutes by using chemiosmotic ion gradients .,similarity:Belongs to the major facilitator superfamily.,tissue specificity:Expressed at very low levels in all tissues tested., |
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