ITT6078
ITT6078
ITT6078
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$424.00
Price in reward points: 424
- Catalog: ITT6078
- Gene/Protein: FA2H
- Product Description: Immunotag™ FA2H Polyclonal Antibody
Available Options
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Description
Immunotag™ FA2H Polyclonal Antibody
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein FA2H Clonality Polyclonal Storage/Stability -20°C/1 year Application WB,ELISA Recommended Dilution WB 1:500-2000, ELISA 1:10000-20000 Concentration 1 mg/ml Reactive Species Human,Mouse,Rat Host Species Rabbit Immunogen Synthesized peptide derived from human FA2H. at AA range: 101-150 Specificity FA2H Polyclonal Antibody detects endogenous levels of FA2H Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Gene Name FA2H Accession No. Q7L5A8 Q5MPP0 Alternate Names Fatty acid 2-hydroxylase (EC 1.-.-.-) (Fatty acid alpha-hydroxylase) Description fatty acid 2-hydroxylase(FA2H) Homo sapiens This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia.[provided by RefSeq, Mar 2010], Protein Expression Colon,Gastric mucosa,Pancreas, Subcellular Localization endoplasmic reticulum,endoplasmic reticulum membrane,integral component of membrane,organelle membrane, Protein Function cofactor:Iron.,disease:Defects in FA2H are the cause of leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia (DLDSP) [MIM:612443]. The disorder consists of a progressive neurologic disease manifested by spasticity, disordered tonicity of muscle, and white matter degeneration.,domain:The histidine box domains may contain the active site and/or be involved in metal ion binding.,function:Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids.,induction:Up-regulated during keratinocyte differentiation.,similarity:Belongs to the SCS7 family.,similarity:Contains 1 cytochrome b5 heme-binding domain.,tissue specificity:Detected in differentiating cultured keratinocytes (at protein level). Detected in epidermis and cultured keratinocytes. Highly expressed in brain and colon. Detected at lower levels in testis, prostate, pancreas and kidney., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | FA2H |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | WB 1:500-2000, ELISA 1:10000-20000 |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from human FA2H. at AA range: 101-150 |
| Specificity | FA2H Polyclonal Antibody detects endogenous levels of FA2H |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | FA2H |
| Accession No. | Q7L5A8 Q5MPP0 |
| Alternate Names | Fatty acid 2-hydroxylase (EC 1.-.-.-) (Fatty acid alpha-hydroxylase) |
| Description | fatty acid 2-hydroxylase(FA2H) Homo sapiens This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia.[provided by RefSeq, Mar 2010], |
| Protein Expression | Colon,Gastric mucosa,Pancreas, |
| Subcellular Localization | endoplasmic reticulum,endoplasmic reticulum membrane,integral component of membrane,organelle membrane, |
| Protein Function | cofactor:Iron.,disease:Defects in FA2H are the cause of leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia (DLDSP) [MIM:612443]. The disorder consists of a progressive neurologic disease manifested by spasticity, disordered tonicity of muscle, and white matter degeneration.,domain:The histidine box domains may contain the active site and/or be involved in metal ion binding.,function:Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids.,induction:Up-regulated during keratinocyte differentiation.,similarity:Belongs to the SCS7 family.,similarity:Contains 1 cytochrome b5 heme-binding domain.,tissue specificity:Detected in differentiating cultured keratinocytes (at protein level). Detected in epidermis and cultured keratinocytes. Highly expressed in brain and colon. Detected at lower levels in testis, prostate, pancreas and kidney., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |