Western Blot (WB)
(Western Blot analysis of mouse-heart cells using primary antibody diluted at 1:2000(4 degree C overnight). Secondary antibody:Goat Anti-rabbit IgG IRDye 800(diluted at 1:5000, 25 degree C, 1 hour))
Rabbit FA2H Polyclonal Antibody | anti-FA2H antibody
FA2H Antibody
ELISA: 1:10000-20000
Western Blot (WB)
(Western Blot analysis of mouse-heart cells using primary antibody diluted at 1:2000(4 degree C overnight). Secondary antibody:Goat Anti-rabbit IgG IRDye 800(diluted at 1:5000, 25 degree C, 1 hour))
Western Blot (WB)
(Western Blot analysis of mouse-heart cells using primary antibody diluted at 1:2000(4 degree C overnight). Secondary antibody:Goat Anti-rabbit IgG IRDye 800(diluted at 1:5000, 25 degree C, 1 hour))
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
Function: Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids. Ref.6 Ref.7
Cofactor: Iron
By similarity.
Subcellular location: Endoplasmic reticulum membrane; Multi-pass membrane protein. Microsome membrane; Multi-pass membrane protein Ref.6.
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. Ref.6 Ref.7
Induction: Up-regulated during keratinocyte differentiation. Ref.7
Domain: The histidine box domains may contain the active site and/or be involved in metal ion binding.
Involvement in disease: Spastic paraplegia 35, autosomal recessive (SPG35) [MIM:612319]: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG35 is a complicated form characterized by childhood onset of gait difficulties. It has a rapid progression and many patients become wheelchair-bound as young adults. Patients manifest cognitive decline associated with leukodystrophy. Other variable neurologic features, such as dystonia, optic atrophy, and seizures may also occur.Note: The disease is caused by mutations affecting the gene represented in this entry.
Sequence similarities: Belongs to the sterol desaturase family. SCS7 subfamily.Contains 1 cytochrome b5 heme-binding domain.
Sequence caution: The sequence AAC23496.1 differs from that shown. Reason: Erroneous gene model prediction.
Research Articles on FA2H
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Product Notes
The FA2H fa2h (Catalog #AAA7122969) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The FA2H Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's FA2H can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB: 1:500-2000 ELISA: 1:10000-20000. Researchers should empirically determine the suitability of the FA2H fa2h for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "FA2H, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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