Did you know ? If you order before Friday 14h we deliver 90PCT of the the time next Tuesday, GENTAUR another in time delivery

Pubmed ID :16971387
Publication Date : //

Dimerization of Laforin is required for its optimal phosphatase activity, regulation of GSK3beta phosphorylation, and Wnt signaling.


Epilepsy of progressive myoclonus type 2 gene A (EPM2A) encodes a dual specificity protein phosphatase called Laforin. Laforin is also a tumor suppressor that dephosphorylates GSK3beta at the critical Ser9 position and regulates Wnt signaling. The epilepsy-causing mutations have a deleterious effect on phosphatase activity, regardless of whether they locate in the carbohydrate-binding domain (CBD) at the N terminus or the dual specificity phosphatase domain (DSPD) at the C terminus. How mutations outside the DSPD reduce the phosphatase activity of Laforin remains unexplained. Here we report that Laforin expressed in mammalian cells forms dimers that are highly resistant to SDS treatment. Deleting CBD completely abolished the dimerization and phosphatase activity of Laforin. Moreover, all of the naturally occurring Laforin mutations tested impaired laforin GSK3beta dephosphorylation at Ser9 dimerization, and beta-catenin accumulation in nucleus. Our results demonstrate a critical role of dimerization in Laforin function and suggest an important new dimension in protein phosphatase function and in molecular pathogenesis of Lafora's disease.

Authors : Liu Yan , Wang Yin , Wu Cindy , Liu Yang , Zheng Pan ,

Related products :

Catalog number Product name Quantity
28-437 ZNF675 may be involved in transcriptional regulation. It may play a role during osteoclast differentiation by modulating TRAF6 signaling activity. 0.1 mg
26-188 DULLARD is a serine_threonine phosphatase which may be required for proper nuclear membrane morphology. DULLARD was involved in LPIN1 dephosphorylation. It may antagonize BMP signaling. 0.05 mg
26-412 DOLPP1 is a multi-pass membrane proteinBy similarity. It belongs to the dolichyldiphosphatase family. It is required for efficient N-glycosylation and is necessary for maintaining optimal levels of do 0.05 mg
30-276 GDE1 has glycerophosphoinositol phosphodiesterase activity. It has little or no activity towards glycerophosphocholine. GDE1 activity can be modulated by G-protein signaling pathways. 0.05 mg
26-900 FES is the human cellular counterpart of a feline sarcoma retrovirus protein with transforming capabilities. FES has tyrosine-specific protein kinase activity and that activity is required for mainten 0.05 mg
29-022 RCOR1 is a functional corepressor required for regulation of neural-specific gene expression.The RCOR gene encodes a functional corepressor required for regulation of neural-specific gene expression.T 0.1 mg
29-023 RCOR1 is a functional corepressor required for regulation of neural-specific gene expression.The RCOR gene encodes a functional corepressor required for regulation of neural-specific gene expression. 0.05 mg
30-156 ATIC is a bifunctional protein requiring dimerization for transformylase activity. 0.1 mg
30-155 ATIC is a bifunctional protein requiring dimerization for transformylase activity. 0.1 mg
AA0038 p53 Signaling Phospho-Specific Array includes 196 highly specific and well-characterized phosphorylation antibodies in the p53 signaling pathway. 2 Pieces/Box
AA0027 GPCR Signaling to MAPK_ERk Phospho-Specific Array includes 193 highly specific and well-characterized phosphorylation antibodies related to this signaling pathway. 2 Pieces/Box
AA0028 IGF-1R Signaling Phospho-Specific Array includes 245 highly specific and well-characterized phosphorylation antibodies in the insulin-like growth factor 1 receptor signaling pathway. 2 Pieces/Box
60511 SRE Reporter Kit (MAPK_ERK Signaling Pathway ),The MAPK_ERK signaling pathway is a major participant in the regulation of cell growth and differentiation. It can be activated by various extracellular 500 reactions
26-614 Deoxycytidine kinase (DCK) is required for the phosphorylation of several deoxyribonucleosides and their nucleoside analogs. Deficiency of DCK is associated with resistance to antiviral and anticancer 0.05 mg
AA0014 AMPK Signaling Phospho-Specific Array includes 174 highly specific and well-characterized phosphorylation antibodies in the AMPK signaling pathway. 2 Pieces/Box
AA0013 AKT_PKB Signaling Phospho-Specific Array includes 216 highly specific and well-characterized phosphorylation antibodies in the AKT_PKB signaling pathway. 2 Pieces/Box
AA0041 TGF-Beta Signaling Phospho-Specific Array includes 176 highly specific and well-characterized phosphorylation antibodies in the TGF-Beta signaling pathway. 2 Pieces/Box
29-805 RGS18 is a member of the regulator of G-protein signaling family. This protein is contains a conserved, 120 amino acid motif called the RGS domain. The protein attenuates the signaling activity of G-p 0.1 mg
AB-004-100 Histone H3 Polyclonal, Rabbit Chromosomal surfaces are ornamented with a variety of posttranslational modifications of histones, which are required for the regulation of many of the DNA_templated proc 100 ul
AA0040 T-Cell Receptor Signaling Phospho-Specific Array includes 188 highly specific and well-characterized phosphorylation antibodies in the T-cell receptor signaling pathway. 2 Pieces/Box
28-991 MYST4 is a histone acetyltransferase which may be involved in both positive and negative regulation of transcription. It is required for RUNX2-dependent transcriptional activation and May be involved 0.05 mg
27-215 CDC25C gene is highly conserved during evolution and it plays a key role in the regulation of cell division. The protein is a tyrosine phosphatase and belongs to the Cdc25 phosphatase family. It direc 0.05 mg
POPN-500 pNPP Phosphatase Assay Kit, Quantitative determination of phosphatase activity by colorimetric (405nm) method 500Tests
POPN-01K pNPP Phosphatase Assay Kit, Quantitative determination of phosphatase activity by colorimetric (405nm) method 1000Tests
29-831 THOC4 is a heat stable, nuclear protein and functions as a molecular chaperone. It is thought to regulate dimerization, DNA binding, and transcriptional activity of basic region-leucine zipper (bZIP) 0.1 mg


 

GENTAUR Belgium BVBA BE0473327336
Voortstraat 49, 1910 Kampenhout BELGIUM
Tel 0032 16 58 90 45

Fax 0032 16 50 90 45
info@gentaur.com | Gentaur





GENTAUR Ltd.
Howard Frank Turnberry House
1404-1410 High Road
Whetstone London N20 9BH
Tel 020 3393 8531 Fax 020 8445 9411
uk@gentaur.com | Gentaur

 

 




GENTAUR France SARL
9, rue Lagrange, 75005 Paris
Tel 01 43 25 01 50

Fax 01 43 25 01 60
RCS Paris B 484 237 888

SIRET 48423788800017

BNP PARIBAS PARIS PL MAUBERT BIC BNPAFRPPPRG

france@gentaur.com | Gentaur

GENTAUR GmbH
Marienbongard 20
52062 Aachen Deutschland
Support Karolina Elandt
Tel: 0035929830070
Fax: (+49) 241 56 00 47 88

Logistic :0241 40 08 90 86
Bankleitzahl 39050000
IBAN lautet DE8839050000107569353
Handelsregister Aachen HR B 16058
Umsatzsteuer-Identifikationsnummer *** DE 815175831
Steuernummer 201/5961/3925
de@gentaur.com | Gentaur

GENTAUR U.S.A
Genprice Inc, Logistics
547, Yurok Circle
San Jose, CA 95123
CA 95123
Tel (408) 780-0908,
Fax (408) 780-0908,
sales@genprice.com

Genprice Inc, Invoices and accounting
6017 Snell Ave, Ste 357
San Jose, CA 95123




GENTAUR Nederland BV
NL850396268B01 KVK nummer 52327027
Kuiper 1
5521 DG Eersel Nederland
Tel:  0208-080893  Fax: 0497-517897
nl@gentaur.com | Gentaur
IBAN: NL04 RABO 0156 9854 62   SWIFT RABONL2U






GENTAUR Spain
tel:0911876558
spain@gentaur.com | Gentaur






ГЕНТАУЪР БЪЛГАРИЯ
ID # 201 358 931 /BULSTAT
София 1000, ул. "Граф Игнатиев" 53 вх. В, ет. 2
Tel 0035924682280 Fax 0035924808322
e-mail: Sofia@gentaur.com | Gentaur
IBAN: BG11FINV91501014771636
BIC: FINVBGSF

GENTAUR Poland Sp. z o.o.


ul. Grunwaldzka 88/A m.2
81-771 Sopot, Poland
TEL Gdansk 058 710 33 44 FAX  058 710 33 48              

poland@gentaur.com | Gentaur

Other countries

Österreich +43720880899

Canada Montreal +15149077481

Ceská republika Praha +420246019719

Danmark +4569918806

Finland Helsset +358942419041

Magyarország Budapest +3619980547

Ireland Dublin+35316526556

Luxembourg+35220880274

Norge Oslo+4721031366

Sverige Stockholm+46852503438

Schweiz Züri+41435006251

US New York+17185132983

GENTAUR Italy
SRL IVA IT03841300167
Piazza Giacomo Matteotti, 6
24122 Bergamo Tel 02 36 00 65 93
Fax 02 36 00 65 94
italia@gentaur.com | Gentaur