N-Acetylgalactosaminidase Alpha (NAGA) Antibody
286€ (100 µl)
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Name
N-Acetylgalactosaminidase Alpha (NAGA) Antibody
Category
Primary Antibodies
Provider
Abbexa
Reference
abx103289
Tested Applications
WB, IHC
Description
Polyclonal Antibody to N-Acetylgalactosaminidase Alpha (NAGa).
Documentos del producto
Instrucciones
Data sheet
Especificaciones del producto
| Category | Primary Antibodies |
| Immunogen Target | Target: N-Acetylgalactosaminidase Alpha (NAGA) Immunogen: NAGa (Leu18-Asp217) |
| Host | Rabbit |
| Reactivity | Mouse |
| Recommended Dilution | WB: 0.01-2 µg/ml, IHC: 5-20 µg/ml, IF/ICC (Predicted): 5-20 µg/ml. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Purification | Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography. |
| Size 1 | 100 µl |
| Size 2 | 200 µl |
| Size 3 | 1 ml |
| Form | Liquid |
| Tested Applications | WB, IHC |
| Buffer | 0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol. |
| Availability | Shipped within 5-7 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9QWR8 |
| Alias | GALB, D22S674 |
| Background | Antibody anti-NAGA |
| Status | RUO |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |
Background
N-Acetylgalactosaminidase Alpha (NAGA) is an enzyme involved in the breakdown of complex carbohydrates. catalyzes the hydrolysis of terminal alpha-N-acetylgalactosaminyl residues in glycoproteins and glycolipids (lysosomes), Deficiency in NAGA enzyme activity leads to a rare genetic disorder known as Schindler disease, which is characterized by the accumulation of certain glycoproteins and glycolipids in lysosomes. Schindler disease can manifest in different forms, including Schindler disease type I and type II, depending on the severity of the enzyme deficiency. Symptoms of Schindler disease can vary widely but may include developmental delay, intellectual disability, seizures, skeletal abnormalities, and other neurological and systemic manifestations. The severity and progression of the disease can also vary, ranging from mild to severe.
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