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Katalogové číslo: (BOSSBS-6639R-HRP)

Dodavatel: Bioss

Popis: On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for BMP7/OP-1 and GDF5.Involvement in disease; Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA). Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbs, and hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers).Defects in BMPR1B are a cause of brachydactyly type A2 (BDA2) [MIM:112600]. Brachydactylies (BDs) are a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. They have been classified on an anatomic and genetic basis into five groups, A to E, including three subgroups (A1 to A3) that usually manifest as autosomal dominant traits. BDA2 was described first in a large Norwegian kindred. BDA2 is caused by mutations in BMPR1B gene and studies demonstrate that these mutations function as dominant negatives in vitro and in vivo.

Měrná jednotka: 1 * 100 µl

Akce

Katalogové číslo: (PRSI27-870)

Dodavatel: ProSci Inc.

Popis: WHSC1 encodes a protein that contains four domains present in other developmental proteins: a PWWP domain, an HMG box, a SET domain, and a PHD-type zinc finger. It is expressed ubiquitously in early development. Wolf-Hirschhorn syndrome (WHS) is a malformation syndrome associated with a hemizygous deletion of the distal short arm of chromosome 4.

Měrná jednotka: 1 * 50 µG

Akce

Katalogové číslo: (PRSI79-622)

Dodavatel: ProSci Inc.

Popis: a maternal imprinting gene. May possess growth suppressor activity. Two major alternatively spliced isoforms have been described. forms. One form, Meg3-proximal (Meg3p), contains exons 1-3. The second form, Meg3-distal (Meg3d) did not contain exons 1-3 and was present in oocytes and in 1- and 2-cell embryos

Měrná jednotka: 1 * 100 µG

Akce

Katalogové číslo: (PRSI28-717)

Dodavatel: ProSci Inc.

Popis: KCNK5 is one of the members of the superfamily of potassium channel proteins containing two pore-forming P domains. KCNK5 is mainly expressed in the cortical distal tubules and collecting ducts of the kidney. The protein is highly sensitive to external pH and this, in combination with its expression pattern, suggests it may play an important role in renal potassium transport.

Měrná jednotka: 1 * 50 µG

Akce

Katalogové číslo: (PRSI55-133)

Dodavatel: ProSci Inc.

Popis: Normal blood lactate is maintained at about 1.5 mM, and little filtered lactate is excreted in urine. Reabsorption of lactate is mediated by the low-affinity Na(+)-coupled lactate transporter SLC5A12 in the initial part of the proximal tubule and by the high-affinity Na(+)-coupled lactate transporter SLC5A8 (MIM 608044) in the distal proximal tubule (Gopal et al., 2007 [PubMed 17692818]).

Měrná jednotka: 1 * 400 µl

New Product Akce

Katalogové číslo: (BSENC-1700-100)

Dodavatel: Biosensis

Popis: The heat shock proteins were discovered, as the name suggests, since they are heavily upregulated when cells are stressed by temperatures above the normal physiological range. They are expressed in unstressed cells also and have a normal function as chaperones, helping other proteins to fold correctly, and are required in much greater amounts if the cell or tissue is stressed by heat. The increased levels are generated transcriptionally under the influence of a powerful transcription factor, the heat shock factor 1 (HSF1). The different heat shock proteins were originally named based on their SDS-PAGE mobility, so HSP27 has an apparent molecular weight of 27kDa. It is an abundant protein even under non-stress conditions and frequently shows up as a major spot on 2 dimensional gels of cells or tissues. It is known to associate with a variety of other proteins such as actin, intermediate filament subunits and ubiquitin and is found both in the cytoplasm and the nucleus of cells. HSP27 can become heavily phosphorylated under the influence of multiple protein kinases particularly as a result of activation of the p38/SAPK pathway. Upregulation of this protein is protective against neurodegenerative diseases at least in certain mouse models (1). Point mutations in the HSP27 gene are associated with two neurological diseases, Charcot-Marie-Tooth disease type 2F and distal hereditary motor neuropathy IIB (2). These diseases are associated with axonal loss apparently following defects in the transport of neurofilaments.

Měrná jednotka: 1 * 100 µl

Akce

Katalogové číslo: (PRSI56-874)

Dodavatel: ProSci Inc.

Popis: The CD320 antigen gene encodes the transcobalamin receptor (TCBLR). Cellular uptake of cobalamin (vitamin B12) is mediated by receptors expressed on the cell surface. Transcobalamin II (TCN2; MIM 613441), a plasma protein secreted by endothelial cells, binds the cobalamin absorbed in the distal ileum and carries 10 to 30% of total circulating cobalamin. CD320 encodes a transcobalamin receptor that binds TCN2-cobalamin at the plasma membrane and internalizes the complex by endocytosis (Quadros et al., 2009 [PubMed 18779389]).

Měrná jednotka: 1 * 400 µl

New Product Akce

Katalogové číslo: (PRSI27-728)

Dodavatel: ProSci Inc.

Popis: DLX5 is a member of a homeobox transcription factor family. DLX5 may play a role in bone development and fracture healing. Mutation in this gene, which is located in a tail-to-tail configuration with another member of the family on the long arm of chromosome 7, may be associated with split-hand/split-foot malformation.This gene encodes a member of a homeobox transcription factor gene family similiar to the Drosophila distal-less gene. The encoded protein may play a role in bone development and fracture healing. Mutation in this gene, which is located in a tail-to-tail configuration with another member of the family on the long arm of chromosome 7, may be associated with split-hand/split-foot malformation.This gene encodes a member of a homeobox transcription factor gene family similiar to the Drosophila distal-less gene. The encoded protein may play a role in bone development and fracture healing. Mutation in this gene, which is located in a tail-to-tail configuration with another member of the family on the long arm of chromosome 7, may be associated with split-hand/split-foot malformation.

Měrná jednotka: 1 * 50 µG

Akce

Katalogové číslo: (PRSI6211)

Dodavatel: ProSci Inc.

Popis: BAPX1 Antibody: BAPX1 is the mammalian homolog of the Drosophila bagpipe homeobox gene and is expressed in the splanchnic mesoderm and embryonic skeleton. It is one of the earliest developmental markers for the sclerotome portion of the somite and the gut mesentery. BAPX1 is required for normal skeletal development; homozygous inactivating mutations in the BAPX1 gene result in spodylo-megaepiphyseal-metaphyseal dysplasia (SMMD). It has also been suggested to play a role in the proper development of the mammalian gut and is required for distal stomach development as part of a BARX1-dependent pathway.

Měrná jednotka: 1 * 100 µG

Akce

Katalogové číslo: (PRSI26-865)

Dodavatel: ProSci Inc.

Popis: CENPP is the component of the CENPA-CAD (nucleosome distal) complex, a complex recruited to centromeres which is involved in assembly of kinetochore proteins, mitotic progression and chromosome segregation. CENPP may be involved in incorporation of newly synthesized CENPA into centromeres via its interaction with the CENPA-NAC complex.CENPP is a subunit of a CENPH (MIM 605607)-CENPI (MIM 300065)-associated centromeric complex that targets CENPA (MIM 117139) to centromeres and is required for proper kinetochore function and mitotic progression (Okada et al., 2006 [PubMed 16622420]).

Měrná jednotka: 1 * 50 µG

Akce

Katalogové číslo: (PRSI30-107)

Dodavatel: ProSci Inc.

Popis: SBDS is a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The protein may function in RNA metabolism. Mutations within its gene are associated with Shwachman-Bodian-Diamond syndrome.This gene encodes a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The encoded protein may function in RNA metabolism. Mutations within this gene are associated with Shwachman-Bodian-Diamond syndrome. An alternative transcript has been described, but its biological nature has not been determined. This gene has a closely linked pseudogene that is distally located.

Měrná jednotka: 1 * 100 µG

Akce

Katalogové číslo: (PRSI30-108)

Dodavatel: ProSci Inc.

Popis: SBDS is a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The protein may function in RNA metabolism. Mutations within its gene are associated with Shwachman-Bodian-Diamond syndrome.This gene encodes a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The encoded protein may function in RNA metabolism. Mutations within this gene are associated with Shwachman-Bodian-Diamond syndrome. An alternative transcript has been described, but its biological nature has not been determined. This gene has a closely linked pseudogene that is distally located.

Měrná jednotka: 1 * 100 µG

Akce

Katalogové číslo: (PRSI33-375)

Dodavatel: ProSci Inc.

Popis: This antibody recognises human blood group A (monofucosyl and difucosyl A antigens with chain types 1, 2, 3, 4, 5, 6) and Forssmann antigen. It is also reactive with the immuno-dominant A trisaccharide. Blood group antigen expression in human colon cancer was studied by means of two monoclonal antibodies of broad anti-A (HE-14) and anti-type 3 and type 4 chain-based A and H (HE-10) specificity. These antigens were proved to re-appear in tumors of the distal colon, the HE-10 antibody reacting more frequently (9 out of 12 samples) than HE-14 (5 out of 12 samples) and frequently with supra-nuclear staining of the cytoplasm probably in those places of the Golgi apparatus where carbohydrate antigens are synthesised. This staining pattern is characteristic of HE-10 in normal colonic mucosa as well. With HE-14, staining was often absent in less differentiated tumors, while HE-10 did react in such tumors. In some cases, these two antibodies gave different staining patterns in parallel sections from the same tissue sample, primarily at the cellular level. Three out of 12 cases showed blood group antigen expression in the mucosa of the distal colon adjacent to the tumor only when HE-10 mAb was used.

Měrná jednotka: 1 * 100 µG

New Product Akce

Katalogové číslo: (PRSI92-581)

Dodavatel: ProSci Inc.

Popis: Cluster of Differentiation 80, also called B7-1, is a member of cell surface immunoglobulin superfamily which plays key, yet distinct roles in the activation of T cells. It is the ligand for two different proteins on the T cell surface: CD28 and CTLA-4. Studies have shown that CTLA-4 binds mostly to CD80. The structure presents two extracellular domains: a membrane distal variable-like domain (IgV) and a membrane proximal Ig constant-like domain (IgC) along with an intracellular domain. Both IgV and IgC consist of anti-parallel beta sandwiches joined by a short linker region. CD80 is mostly expressed on the surface of antigen-presenting cells including activated B cells, macrophages and dendritic cells.

Měrná jednotka: 1 * 50 µG

Akce

Katalogové číslo: (PRSI33-665)

Dodavatel: ProSci Inc.

Popis: Recognises protein of 192 kDa, which is identified as Clathrin Heavy Chain. Clathrin is composed of three heavy chains and three light chains, which associate non-covalently to form a triskelion structure. Clathrin heavy chain (HC) is composed of a terminal globular domain, a distal segment and a proximal segment containing a light chain-binding site. The proximal segment of the Clathrin HC protein is essential for interactions between Clathrin heavy chains and light chains, which result in the formation of the triskelion structure.

Měrná jednotka: 1 * 100 µG

New Product Akce

Katalogové číslo: (PRSI5639)

Dodavatel: ProSci Inc.

Popis: CAZIP Antibody: CAZIP, also known as TIP150, contains a leucine-zipper domain at the C-terminus and has at least three isoforms. CAZIP is expressed in heart during early cardiac development and in parts of the nervous system in later embryonic development. It is thought to bind microtubules and facilitate the end-binding protein 1 (EB1)-dependent loading of MCAK onto microtubules plus ends and orchestrates the dynamics at the plus end of microtubules. CAZIP may regulate the dynamics of microtubules at their growing distal tip.

Měrná jednotka: 1 * 100 µG

Akce