Rabbit anti-Human NAGA Polyclonal Antibody | anti-NAGA antibody

NAGA (Alpha-N-acetylgalactosaminidase, Alpha-galactosidase B) APC

Cat. Num. AAA6386447

• Gene Names: NAGA; GALB; D22S674
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NAGA; Polyclonal Antibody; NAGA (Alpha-N-acetylgalactosaminidase; Alpha-galactosidase B) APC; anti-NAGA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human NAGA.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-NAGA antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human NAGA, aa1-411 (NP_000253.1).
• Immunogen Sequence: MLLKTVLLLGHVAQVLMLDNGLLQTPPMGWLAWERFRCNINCDEDPKNCISEQLFMEMADRMAQDGWRDMGYTYLNIDDCWIGGRDASGRLMPDPKRFPHGIPFLADYVHSLGLKLGIYADMGNFTCMGYPGTTLDKVVQDAQTFAEWKVDMLKLDGCFSTPEERAQGYPKMAAALNATGRPIAFSCSWPAYEGGLPPRVNYSLLADICNLWRNYDDIQDSWWSVLSILNWFVEHQDILQPVAGPGHWNDPDMLLIGNFGLSLEQSRAQMALWTVLAAPLLMSTDLRTISAQNMDILQNPLMIKINQDPLGIQGRRIHKEKSLIEVYMRPLSNKASALVFFSCRTDMPYRYHSSLGQLNFTGSVIYEAQDVYSGDIISGLRDETNFTVIINPSGVVMWYLYPIKNLEMSQQ
• Conjugate: APC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(NAGA rabbit polyclonal antibody. Western Blot analysis of NAGA expression in human liver.)

Western Blot (WB)

(Western Blot analysis of NAGA expression in transfected 293T cell line by NAGA polyclonal antibody. Lane 1: NAGA transfected lysate (46.6kD). Lane 2: Non-transfected lysate)

Related Product Information for anti-NAGA antibody

NAGA is a lysosomal a-N-acetylgalactosaminidase that cleaves non-reducing a-N-acetylgalactosaminyl moieties from glycoconjugates. Mature NAGA has 394aa and is trafficked to the lysosome via the mannose-6-phosphate receptor-mediated pathway. The enzyme is a retaining exoglycosidase, where both the substrate and product of the enzymatic reaction have the same anomeric configuration. Deficiency in NAGA results in increased urinary excretion and tissue accumulation of glycopeptides and oligosaccharides containing terminal aNacetylgalactosaminyl moieties, manifesting as Schindler's disease, an autosomal recessive disease with neuroaxonal dystrophy and other neurological symptoms. The enzyme can be used to remove a-N-acetylgalactosaminyl residues present on red blood cells thus converting blood type A to blood type O.

Product Categories/Family for anti-NAGA antibody

Antibodies; Enzymes; Glycosidase

NCBI and Uniprot Product Information

• NCBI GI #: 4557781
• NCBI GeneID: 4668
• NCBI Accession #: NP_000253.1
• NCBI GenBank Nucleotide #: NM_000262.1
• UniProt Accession #: P17050
• Molecular Weight: 46,565 Da
• NCBI Official Full Name: alpha-N-acetylgalactosaminidase
• NCBI Official Synonym Full Names: alpha-N-acetylgalactosaminidase
• NCBI Official Symbol: NAGA
• NCBI Official Synonym Symbols: GALB; D22S674
• NCBI Protein Information: alpha-N-acetylgalactosaminidase
• UniProt Protein Name: Alpha-N-acetylgalactosaminidase
• Protein Family: Alpha-N-acetylgalactosaminidase
• UniProt Gene Name: NAGA

NCBI Description

NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). [provided by RefSeq, Jul 2008]

Uniprot Description

NAGA: Removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids. Defects in NAGA are the cause of Schindler disease (SCHIND). Schindler disease is a form of NAGA deficiency characterized by early onset neuroaxonal dystrophy and neurological signs (convulsion during fever, epilepsy, psychomotor retardation and hypotonia). NAGA deficiency is typically classified in three main phenotypes: NAGA deficiency type I (Schindler disease or Schindler disease type I) with severe manifestations; NAGA deficiency type II (Kanzazi disease or Schindler disease type II) which is mild; NAGA deficiency type III (Schindler disease type III) characterized by mild-to-moderate neurologic manifestations. NAGA deficiency results in the increased urinary excretion of glycopeptides and oligosaccharides containing alpha-N-acetylgalactosaminyl moieties. Inheritance is autosomal recessive. Defects in NAGA are the cause of Kanzaki disease (KANZD); also known as NAGA deficiency type II or Schindler disease type II. Kanzaki disease is an autosomal recessive disorder characterized by late onset, angiokeratoma corporis diffusum and mild intellectual impairment. Belongs to the glycosyl hydrolase 27 family.

Protein type: EC 3.2.1.49; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase

Chromosomal Location of Human Ortholog: 22q13.2

Cellular Component: cytoplasm

Molecular Function: alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity; protein homodimerization activity

Biological Process: carbohydrate catabolic process; glycolipid catabolic process; glycoside catabolic process

Disease: Kanzaki Disease; Schindler Disease, Type I