Anti-NAGLU Antibody (CAB20317)
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주문- SKU:
- CAB20317
- Product type:
- Antibody
- Antibody Type:
- Polyclonal Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- NAG
- Synonyms:
- CMT2V
- Synonyms:
- MPS3B
- Synonyms:
- UFHSD
- Synonyms:
- MPS-IIIB
Frequently bought together:
Description
| Product Name: | NAGLU Rabbit pAb |
| Product Code: | CAB20317 |
| Size: | 50uL, 100uL |
| Synonyms: | NAG, CMT2V, MPS3B, UFHSD, MPS-IIIB |
| Applications: | WB |
| Reactivity: | Human |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 350-600 of human NAGLU (NP_000254.2). |
| Applications: | WB |
| Recommended Dilutions: | WB 1:500 - 1:2000 |
| Reactivity: | Human |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 350-600 of human NAGLU (NP_000254.2). |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 4669 |
| Uniprot: | P54802 |
| Calculated MW: | 82kDa |
| Observed MW: | Refer to figures |
| UniProt Protein Function: | NAGLU: Involved in the degradation of heparan sulfate. Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B); also known as Sanfilippo syndrome B. MPS3B is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosaminoglycan degradation; EC 3.2.1.50; Hydrolase Molecular Function:alpha-N-acetylglucosaminidase activity Biological Process: locomotor rhythm; retinal rod cell development; lysosome organization and biogenesis; inner ear receptor cell development; cerebellar Purkinje cell layer development; middle ear morphogenesis |
| UniProt Code: | P54802 |
| NCBI GenInfo Identifier: | 2660688 |
| NCBI Gene ID: | 27419 |
| NCBI Accession: | AAB88084.1 |
| Molecular Weight: | ~81kD |
| NCBI Full Name: | Naglu |
| NCBI Synonym Full Names: | alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB) |
| NCBI Official Symbol: | Naglu |
| NCBI Protein Information: | alpha-N-acetylglucosaminidase; N-acetyl-glucosaminidase; alpha-N-acetylglucosaminidase, lysosomal |
| UniProt Protein Name: | |
| Protein Family: | Alpha-N-acetylglucosaminidase |
| UniProt Gene Name: |
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