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Anti-NAGLU Antibody (CAB20317)

SKU:
CAB20317
Product Type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
NAG
CMT2V
MPS3B
UFHSD
MPS-IIIB
€359
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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