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A Affected person with Berardinelli-Seip Syndrome, Novel AGPAT2 Splicesite Mutation and Concomitant Improvement of Non-diabetic Polyneuropathy

 

  • Main polyneuropathy within the context of Seip-Berardinelli kind 1 seipinopathy, or congenital generalized lipodystrophy kind 1 (CGL1) has not been beforehand reported.

 

  • We report the case historical past of a 27 yr outdated feminine CGL1 affected person presenting with an uncommon further growth of non-diabetic peripheral neuropathy and studying disabilities in early adolescence. Entire exome sequencing (WES) of the affected person genome recognized a novel variant, homozygous for a 52 bp intronic deletion within the AGPAT2 locus, coding for 1-acylglycerol-3-phosphate O-acyltransferase 2, which is uniquely related to CGL1 seipinopathies, with no molecular proof for twin analysis.

        Mouse Leukemia Inhibitory Issue (LIF) ELISA Equipment

  • Purposeful research utilizing RNA remoted from affected person peripheral blood leucocytes confirmed irregular RNA splicing ensuing within the lack of 25 amino acids from the affected person AGPAT2 protein coding sequence. Stability and transcription ranges for the misspliced AGPAT2 mRNA in our affected person nonetheless remained regular.

 

  • Any AGPAT2 protein produced in our affected person is due to this fact more likely to be dysfunctional. Nonetheless, formal linkage of this deletion to the neuropathy noticed stays to be proven.

    Anti-Apaf-1 (human) Monoclonal Antibody (2E12)

  • The classical medical presentation of a affected person with AGPAT2-associated lipodystrophy reveals regular cognition and no growth of polyneuropathy. Cognitive disabilities and polyneuropathy are options related completely with medical CGL kind 2 arising from seipin (BSCL2) gene mutations. This case research means that in some genetic contexts, AGPAT2 mutations may also produce phenotypes with major polyneuropathy.

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Mu-48T 48T
EUR 489
  • Should the Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Mu-96T 96T
EUR 635
  • Should the Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Mu-48Tests 48 Tests
EUR 511

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Mu-96Tests 96 Tests
EUR 709

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Mu-48Tests 48 Tests
EUR 489

Mouse Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Mu-96Tests 96 Tests
EUR 677

anti-LIF
YF-PA12952 50 ug
EUR 363

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-b-48T 48T
EUR 547
  • Should the Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-b-96T 96T
EUR 715
  • Should the Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-c-48T 48T
EUR 527
  • Should the Canine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-c-96T 96T
EUR 688
  • Should the Canine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Hu-48T 48T
EUR 380
  • Should the Human Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Hu-96T 96T
EUR 485
  • Should the Human Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-p-48T 48T
EUR 547
  • Should the Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-p-96T 96T
EUR 715
  • Should the Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Ra-48T 48T
EUR 508
  • Should the Rat Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
DLR-LIF-Ra-96T 96T
EUR 661
  • Should the Rat Leukemia Inhibitory Factor (LIF) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement.

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-b-48Tests 48 Tests
EUR 580

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-b-96Tests 96 Tests
EUR 807

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-c-48Tests 48 Tests
EUR 557

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-c-96Tests 96 Tests
EUR 774

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Hu-48Tests 48 Tests
EUR 383

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Hu-96Tests 96 Tests
EUR 525

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-p-48Tests 48 Tests
EUR 580

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-p-96Tests 96 Tests
EUR 807

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Ra-48Tests 48 Tests
EUR 534

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
RDR-LIF-Ra-96Tests 96 Tests
EUR 742

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-b-48Tests 48 Tests
EUR 555

Bovine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-b-96Tests 96 Tests
EUR 771

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-c-48Tests 48 Tests
EUR 533

Canine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-c-96Tests 96 Tests
EUR 740

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Hu-48Tests 48 Tests
EUR 367

Human Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Hu-96Tests 96 Tests
EUR 502

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-p-48Tests 48 Tests
EUR 555

Porcine Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-p-96Tests 96 Tests
EUR 771

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Ra-48Tests 48 Tests
EUR 511

Rat Leukemia Inhibitory Factor (LIF) ELISA Kit
RD-LIF-Ra-96Tests 96 Tests
EUR 709

Anti-LIF Antibody
A01400 100ug/vial
EUR 294

anti- LIF antibody
FNab09804 100µg
EUR 548.75
  • Recommended dilution: WB: 1:200-1:1000
  • IHC: 1:50-1:500
  • Immunogen: leukemia inhibitory factor
  • Uniprot ID: P15018
  • Research Area: Signal Transduction, Immunology, Developmental biology, Neuroscience, Stem Cells

Anti-LIF Antibody
PA1562 100ug/vial
EUR 294

anti- LIF antibody
LSMab09804 100 ug
EUR 386

Anti-LIF Antibody
PB9036 100ug/vial
EUR 334

Anti-LIF antibody
STJ73631 100 µg
EUR 359

Anti-LIF antibody
STJ29846 100 µl
EUR 277

Anti-LIF antibody
STJ96472 200 µl
EUR 197

Rabbit Polyclonal antibody Anti-CRBN
Anti-CRBN 50 µg
EUR 349

LIF, Mouse
HY-P7084 50ug
EUR 533

LIF, mouse
MO16102 100 ug
EUR 675

Anti-LIF (aa28-39) antibody
STJ72919 100 µg
EUR 359

Mouse LIF shRNA Plasmid
20-abx971321
  • EUR 801.00
  • EUR 1121.00
  • 150 µg
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  • Shipped within 15-20 working days.

Mouse LIF ELISA Kit
EML0014 96Tests
EUR 521

Recombinant Mouse LIF Protein
R01400-1 100ug/vial
EUR 294

Polyclonal Goat anti-GST α-form
GST-ANTI-1 50 uL
EUR 280

Polyclonal Goat anti-GST μ-form
GST-ANTI-2 50 uL
EUR 280

Polyclonal Goat anti-GST p-form
GST-ANTI-3 50 uL
EUR 280

LIF protein
30R-2771 10 ug
EUR 369

LIF protein
30R-2772 10 ug
EUR 489

LIF Antibody
25283-100ul 100ul
EUR 390

LIF antibody
70R-12299 100 ug
EUR 403

LIF antibody
70R-14100 100 ug
EUR 322

Novel Design of Eco-Pleasant Tremendous Elastomer Supplies With Optimized Arduous Segments Micro-Construction: Towards Subsequent-Technology Excessive-Efficiency Tires.

 

  • Not too long ago, sustainable growth has develop into a major concern globally, and the power disaster is among the prime priorities. From the attitude of the commercial software of polymeric supplies, rubber tires are critically necessary in our each day lives. Nonetheless, the power consumption of tires can attain 6% of the world’s whole power consumption each year.

 

  • In the meantime, it’s calculated that round 5% of carbon dioxide comes from the emission of tire rolling as a consequence of power consumption. To beat these extreme power and environmental challenges, designing and growing a high-performance fuel-saving tire is of paramount significance. Herein, a next-generation, eco-friendly tremendous elastomer materials based mostly on macromolecular meeting expertise has been fabricated.

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  • Hydroxyl-terminated solution-polymerized styrene-butadiene rubber (HTSSBR) with excessive vinyl contents ready by anionic polymerization is used as versatile tender segments to acquire glorious moist skid resistance. Moreover, extremely symmetrical 1,5-naphthalene diisocyanate (NDI), completely different proportions of chain extender, and the cross-linking agent with average molecular size are chosen as inflexible laborious segments to realize simultaneous excessive warmth resistance.

 

  • By way of this strategy, a homogeneous community supported by uniformly distributed laborious section nanoparticles is fashioned as a result of tender segments with equal size are chemically end-linked by the laborious segments. This super elastomer materials displays glorious put on resistance and low rolling resistance.

          Anti-Cytokeratin 1 Rabbit Monoclonal Antibody

  • Extra importantly, the wear and tear resistance, rolling resistance, and wet-skid resistance are diminished by 85.4, 42.3, and 20.8%, respectively, in comparison with the elastomeric materials conventionally used for tire. By profiting from this glorious complete service efficiency, the long-standing problem of the “magic triangle” plaguing the rubber tire business for nearly 100 years is resolved. It’s anticipated that this newly designed and fabricated elastomeric materials tailor-made for tires will develop into the subsequent era product, which may exhibit excessive potential for considerably reducing the gas consumption and decreasing the emission of carbon dioxide.

Anti-ALK Rabbit Monoclonal Antibody, Clone#RM361
M00301-1 100uL
EUR 385

Polyclonal Goat anti-GST α-form
GST-ANTI-1 50 uL
EUR 280

Anti-Periphilin 1 Antibody
A06996-1 100ul
EUR 397

Anti-Lyl-1 Antibody
A07491-1 100ul
EUR 397

Anti-GLI-1 Antibody
A07972-1 100ul
EUR 397

Anti-Cerebellin 1 Antibody
A09176-1 100ul
EUR 397

Anti-DOC-1 Antibody
A09467-1 100ul
EUR 397

Anti-NPDC-1 Antibody
A12846-1 100ul
EUR 397

Anti-CNG-1 Antibody
A05494-1 100ul
EUR 397

Anti-Flk-1 Antibody
A00901-1 100ul
EUR 397

Anti-EDG-1 Antibody
A01502-1 100ul
EUR 397

Anti-ROBO-1 Antibody
A01530-1 100ug
EUR 455

Anti-Bag-1 Antibody
A02423-1 50 ul
EUR 397

Anti-TUB 1 Antibody
A02917-1 100ug/vial
EUR 294

Anti-Dok-1 Antibody
A03039-1 100ul
EUR 397

Anti-TFIIIB90-1 Antibody
A03761-1 100ul
EUR 397

Anti-Atrophin-1 Antibody
A03828-1 100ul
EUR 397

Anti-PARP-1 Antibody
A00122-1 100ul
EUR 397

Anti-Presenilin 1 Antibody
A00138-1 100ul
EUR 397

Anti-PAI-1 Antibody
A00637-1 100ul
EUR 397

Anti-ALK-1 antibody
STJ91563 200 µl
EUR 197

Anti-ALK-1 antibody
STJ96547 200 µl
EUR 197

Anti-Apaf-1 (human) Monoclonal Antibody (2E12)
M00889-1 100ug
EUR 432

Anti-HO-1 Monoclonal Antibody (HO-1-2)
M00253-1 1mg
EUR 397

Anti-Pyrophosphatase 1/PPA1 Antibody
A07485-1 100ug/vial
EUR 334

Anti-TCP-1 eta Antibody
A08169-1 100ul
EUR 397

Anti-Relaxin 1/RLN1 Antibody
A08367-1 100ug/vial
EUR 334

Anti-TCP-1 zeta Antibody
A09373-1 100ul
EUR 397

Anti-CHST8/Galnac4St 1 Antibody
A10989-1 100ul
EUR 397

Anti-Musashi 1/Msi1 Antibody
A05052-1 100ug/vial
EUR 334

Anti-LOX-1/OLR1 Antibody
A00760-1 100ug/vial
EUR 294

Anti-Syndecan-1/SDC1 Antibody
A00991-1 100ug/vial
EUR 294

Anti-IRAK-1/IRAK1 Antibody
A01021-1 100ug/vial
EUR 334

Anti-Neurexin 1/NRXN1 Antibody
A01490-1 100ug/vial
EUR 334

Anti-Hexokinase 1/HK1 Antibody
A01504-1 100ug/vial
EUR 334

Anti-Synaptotagmin 1/SYT1 Antibody
A02314-1 100ug/vial
EUR 334

Anti-Syntenin-1 Monoclonal Antibody
A02475-1 100ul
EUR 397

Anti-Dsg1/Desmoglein 1 Antibody
A02655-1 100ul
EUR 397

Anti-CAF-1 p150 Antibody
A02732-1 100ul
EUR 397

Anti-Talin 1/TLN1 Antibody
A02859-1 100ug/vial
EUR 334

Anti-Islet 1/ISL1 Antibody
A02969-1 100ug/vial
EUR 334

Anti-TNF Receptor 1 Antibody
A00294-1 200ug
EUR 522

Anti-Galectin 1/Lgals1 Antibody
A00470-1 100ug/vial
EUR 334

Anti-Angiopoietin 1/ANGPT1 Antibody
PA1333-1 100ug/vial
EUR 334

Anti-Presenilin 1 Monoclonal Antibody
M00138-1 100ug
EUR 397

Anti-Galectin 1 Monoclonal Antibody
M00470-1 100ug
EUR 397

Anti-DJ-1 Monoclonal Antibody
M00757-1 100ul
EUR 397

Anti-Enolase 1 Monoclonal Antibody
M01250-1 100ul
EUR 397

Anti-Dynamin 1 Monoclonal Antibody
M02536-1 100ug
EUR 397

Anti-Esrp-1 Monoclonal Antibody
M06068-1 100uL
EUR 443

Anti-Galectin 1/LGALS1 Antibody
PB9240-1 100ug/vial
EUR 334

Anti-P-glycoprotein (human) Monoclonal Antibody (JSB-1)
M00049-1 125ug
EUR 586

AXMIR-1 RNA oligo anti-miRNA-1-3p with Xmotif
AXMIR-1 10 reactions
EUR 438
  • Category: Exosome

Anti-BOB-1/OBF-1 Rabbit Monoclonal Antibody, Clone#RM378
M04431-1 100uL
EUR 385

Anti-CELSR3/Flamingo Homolog 1 Antibody
A07204-1 100ul
EUR 397

Anti-LPCAT2/Acyltransferase Like 1 Antibody
A07471-1 100ul
EUR 397

Anti-Hyaluronan synthase 1/HAS1 Antibody
A04784-1 100ug/vial
EUR 334

Anti-Liver Carboxylesterase 1/CES1 Antibody
A01741-1 100ug/vial
EUR 334

Anti-EBP50/NHERF-1/SLC9A3R1 Antibody
A02427-1 100ug/vial
EUR 334

Anti-IL1R2/Il 1 Rii Antibody
A03106-1 1 ml
EUR 397

Anti-HIF-1 alpha/HIF1A Antibody
A00013-1 100ug/vial
EUR 294

Anti-Cleaved-Notch 1 (V1754) Antibody
A00033-1 100ul
EUR 397

Anti-HDAC-1 (C-terminus) Antibody
A00256-1 100uL
EUR 443

Anti-VEGF Receptor 1/FLT1 Antibody
A00534-1 100ug/vial
EUR 294

Anti-Integrin alpha 1/ITGA1 Antibody
PA1045-1 100ug/vial
EUR 334

Anti-Caspase-1(P20)/CASP1 Antibody
PA1440-1 100ug/vial
EUR 294

Anti-VEGF Receptor 1/FLT1 Antibody
PA1966-1 100ug/vial
EUR 294

Anti-Sumo 1 Rabbit Monoclonal Antibody
M00631-1 100ug/vial
EUR 397
  • netheless remained regular. Any AGPAT2 protein produced in our affected person is due to this fact more likely to be dysfunctional. Nonetheless, formal linkage of this deletion to the neuropathy noticed stays to be proven. The classical medical presentation of a affected person with AGPAT2-associated lipodystrophy reveals regular cognition and no development of polyneuropathy. Cognitive disabilities and pol

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