Epilepsy, Progressive Myoclonus Type 2A (Lafora Disease, EPM2A, Laforin, LD, LDE, EPM2, MELF, LAFPTPase, FLJ11207, KIAA0766)

Katalog-Nummer E3384-75D-100ug

Size : 100ug

Marke : US Biological



E3384-75D Epilepsy, Progressive Myoclonus Type 2A (Lafora Disease, EPM2A, Laforin, LD, LDE, EPM2, MELF, LAFPTPase, FLJ11207, KIAA0766)

Clone Type
Polyclonal
Host
goat
Source
human
Isotype
IgG
Grade
Affinity Purified
Applications
E IHC WB
Crossreactivity
Hu
Accession #
NP_005661.1
Shipping Temp
Blue Ice
Storage Temp
-20°C

Laforin is encoded by the EMP2A gene and plays a critical role in the survival of neurons in the brain. Laforin is also expressed in many other cells throughout the body and has mutliple functions. To carry out these functions, laforin interacts with several other proteins, including malin (which is produced from the NHLRC1 gene). These proteins are part of complex networks that transmit chemical signals and break down unneeded or abnormal proteins. Mutations in the EPM2A gene lead to the major features of Lafora progressive myoclonus epilepsy. Additionally, laforin may act as a tumor suppressor protein.

Applications:
Suitable for use in ELISA, Western Blot, Immunohistochemistry. Other applications not tested.

Recommended Dilution:
ELISA: 1:6000
Western Blot: 0.1-0.3ug/ml. ~38kD band observed in Human Brain (Cerebellum), Mouse Brain and Rat Brain lysates (MW 37.2kD).
Immunohistochemistry(Paraffin): 2.5-3.8ug/ml
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Applications
Product Type: Pab|Isotype: IgG|Host: goat|Source: human|Concentration: ~0.5mg/ml|Form: Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.|Purity: Purified by affinity chromatography.|Immunogen: Synthetic peptide corresponding to C-EATGHTNEMKHTTD of human Laforin at the internal region. Species sequence homology: mouse, rat, canine and bovine.|Specificity: Recognizes human EPM2A.||Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Immunogen
Synthetic peptide corresponding to C-EATGHTNEMKHTTD of human Laforin at the internal region. Species sequence homology: mouse, rat, canine and bovine.
Form
Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.
Purity
Purified by affinity chromatography.
Specificity
Recognizes human EPM2A.
References
Mittal S, Dubey D, Yamakawa K, Ganesh S.|Lafora disease proteins malin and laforin are recruited to aggresomes in response to proteasomal impairment.|Hum Mol Genet. 2007 Apr 1;16(7):753-62. Epub 2007 Mar 2.|PMID: 17337485|