Western Blot (WB)
(Western blot analysis of lysates from HepG2, A431, A549 cell line and human brain tissue lysate(from left to right), using SERAC1 Antibody (N-term). MBS9211043 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:5000 dilution was used as the secondary antibody. Lysates at 35ug per lane.)
Rabbit anti-Human SERAC1 Polyclonal Antibody | anti-SERAC1 antibody
SERAC1 Antibody (N-term)
Western Blot (WB)
(Western blot analysis of lysates from HepG2, A431, A549 cell line and human brain tissue lysate(from left to right), using SERAC1 Antibody (N-term). MBS9211043 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:5000 dilution was used as the secondary antibody. Lysates at 35ug per lane.)
Western Blot (WB)
(Western blot analysis of lysates from HepG2, A431, A549 cell line and human brain tissue lysate(from left to right), using SERAC1 Antibody (N-term). MBS9211043 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:5000 dilution was used as the secondary antibody. Lysates at 35ug per lane.)
Mungall A.J.,et al.Nature 425:805-811(2003).
Tort F.,et al.Mol. Genet. Metab. 110:73-77(2013).
Wortmann S.B.,et al.Nat. Genet. 44:797-802(2012).
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is a phosphatidylglycerol remodeling protein found at the interface of mitochondria and endoplasmic reticula, where it mediates phospholipid exchange. The encoded protein plays a major role in mitochondrial function and intracellular cholesterol trafficking. Defects in this gene are a cause of 3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome (MEGDEL). Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Aug 2012]
Uniprot Description
SERAC1: Plays an important role in the phosphatidylglycerol remodeling that is essential for both mitochondrial function and intracellular cholesterol trafficking. May catalyze the remodeling of phosphatidylglycerol and be involved in the transacylation- acylation reaction to produce phosphatidylglycerol-36:1. May be involved in bis(monoacylglycerol)phosphate biosynthetic pathway. Defects in SERAC1 are the cause of 3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome (MEGDEL). An autosomal recessive disorder characterized by childhood onset of delayed psychomotor development or psychomotor regression, sensorineural deafness, spasticity or dystonia, and increased excretion of 3- methylglutaconic acid. Brain imaging shows cerebral and cerebellar atrophy as well as lesions in the basal ganglia reminiscent of Leigh syndrome. Laboratory studies show increased serum lactate and alanine, mitochondrial oxidative phosphorylation defects, abnormal mitochondria, abnormal phosphatidylglycerol and cardiolipin profiles in fibroblasts, and abnormal accumulation of unesterified cholesterol within cells. Belongs to the SERAC1 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, integral
Chromosomal Location of Human Ortholog: 6q25.3
Cellular Component: nucleoplasm; extracellular matrix; mitochondrion; endoplasmic reticulum; cytoplasm; integral to membrane
Molecular Function: hydrolase activity, acting on ester bonds
Biological Process: intracellular protein transport; extracellular matrix organization and biogenesis; GPI anchor metabolic process; phospholipid biosynthetic process
Disease: 3-methylglutaconic Aciduria With Deafness, Encephalopathy, And Leigh-like Syndrome
Research Articles on SERAC1
Similar Products
Product Notes
The SERAC1 serac1 (Catalog #AAA9211043) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The SERAC1 Antibody (N-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's SERAC1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the SERAC1 serac1 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 "SERAC1, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
