NAGLU Antibody (54-G): sc-130383

NAGLU Antibody (54-G) is a mouse monoclonal IgG2a kappa light chain antibody that detects NAGLU protein of mouse, rat, and human origin by western blotting (WB) and immunoprecipitation (IP). Anti-NAGLU antibody (54-G) is available as the non-conjugated form. NAGLU, or N-acetyl-alpha-glucosaminidase, is a 743 amino acid protein that plays a crucial role in the degradation of heparan sulfate, specifically catalyzing the hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides. This enzymatic function is vital for maintaining cellular homeostasis, as heparan sulfate accumulation due to NAGLU deficiency can lead to severe metabolic disorders, including mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. MPS-IIIB is an autosomal recessive disorder characterized by the body′s inability to properly degrade heparan sulfate, resulting in accumulation in lysosomes and urine, which ultimately leads to progressive mental deterioration and other serious health complications. NAGLU is expressed in various tissues, including ovary, liver, testis, prostate, lung, colon, kidney, spleen, placenta, and peripheral blood leukocytes, highlighting its importance in multiple biological processes.

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NAGLU Antibody (54-G) References:

1. Sanfilippo type B syndrome (mucopolysaccharidosis III B): allelic heterogeneity corresponds to the wide spectrum of clinical phenotypes.  |  Weber, B., et al. 1999. Eur J Hum Genet. 7: 34-44. PMID: 10094189
2. Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: Diagnostic, clinical, and biological implications.  |  Yogalingam, G. and Hopwood, JJ. 2001. Hum Mutat. 18: 264-81. PMID: 11668611
3. Sanfilippo type B syndrome: five patients with an R565P homozygous mutation in the alpha-N-acetylglucosaminidase gene from the Okinawa islands in Japan.  |  Chinen, Y., et al. 2005. J Hum Genet. 50: 357-359. PMID: 15933803
4. Development of sensory, motor and behavioral deficits in the murine model of Sanfilippo syndrome type B.  |  Heldermon, CD., et al. 2007. PLoS One. 2: e772. PMID: 17712420
5. Structural and mechanistic insight into the basis of mucopolysaccharidosis IIIB.  |  Ficko-Blean, E., et al. 2008. Proc Natl Acad Sci U S A. 105: 6560-5. PMID: 18443291
6. Differential Uptake of NAGLU-IGF2 and Unmodified NAGLU in Cellular Models of Sanfilippo Syndrome Type B.  |  Prill, H., et al. 2019. Mol Ther Methods Clin Dev. 14: 56-63. PMID: 31309128
7. Downregulation of NAGLU in VEC Increases Abnormal Accumulation of Lysosomes and Represents a Predictive Biomarker in Early Atherosclerosis.  |  Xing, C., et al. 2021. Front Cell Dev Biol. 9: 797047. PMID: 35155448
8. Early-onset motor polyneuropathy associated with a novel dominant NAGLU mutation.  |  Lopergolo, D., et al. 2023. Neurol Sci. 44: 1415-1418. PMID: 36648562
9. The molecular basis of Sanfilippo syndrome type B.  |  Zhao, HG., et al. 1996. Proc Natl Acad Sci U S A. 93: 6101-5. PMID: 8650226
10. Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B).  |  Weber, B., et al. 1996. Hum Mol Genet. 5: 771-7. PMID: 8776591
11. NAGLU mutations underlying Sanfilippo syndrome type B.  |  Schmidtchen, A., et al. 1998. Am J Hum Genet. 62: 64-9. PMID: 9443878
12. Mucopolysaccharidosis type IIIB (Sanfilippo B): identification of 18 novel alpha-N-acetylglucosaminidase gene mutations.  |  Bunge, S., et al. 1999. J Med Genet. 36: 28-31. PMID: 9950362

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-NAGLU Antibody (54-G) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-NAGLU Antibody (54-G) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

NAGLU Antibody (54-G) Specifications

Alternate Product Name: NAGLU

Product Clone Name: 54-G

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 644-743

Epitope Target: NAGLU

Epitope Species: human

Product Reactivity/Detection: NAGLU

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP

Vialing Information: Each vial contains 100 µg IgG_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for NAGLU Antibody (54-G): N/A