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Supplier: Biotium
Description: This MAb reacts with a 68 kDa protein, identified as light sub-unit of neurofilaments (NF-L). Neurofilaments make up the main structural elements of axons and dendrites and are found in neurons, peripheral nerves, and sympathetic ganglion cells. Neurofilaments consist of three major subunits with molecular weights of 68 kDa (NF-L), 160 kDa (NF-M) and 200 kDa (NF-H). Anti-neurofilament stains a number of neural, neuroendocrine, and endocrine tumors. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas, and neuroblastomas stain positively for anti-neurofilament. Neurofilaments are also present in paragangliomas as well as adrenal and extra-adrenal pheochromocytomas. Carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung also express neurofilament.

Supplier: Biotium
Description: This MAb reacts with a 68 kDa protein, identified as light sub-unit of neurofilaments (NF-L). Neurofilaments make up the main structural elements of axons and dendrites and are found in neurons, peripheral nerves, and sympathetic ganglion cells. Neurofilaments consist of three major subunits with molecular weights of 68 kDa (NF-L), 160 kDa (NF-M) and 200 kDa (NF-H). Anti-neurofilament stains a number of neural, neuroendocrine, and endocrine tumors. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas, and neuroblastomas stain positively for anti-neurofilament. Neurofilaments are also present in paragangliomas as well as adrenal and extra-adrenal pheochromocytomas. Carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung also express neurofilament.

Supplier: Biotium
Description: This MAb reacts with a 68 kDa protein, identified as light sub-unit of neurofilaments (NF-L). Neurofilaments make up the main structural elements of axons and dendrites and are found in neurons, peripheral nerves, and sympathetic ganglion cells. Neurofilaments consist of three major subunits with molecular weights of 68 kDa (NF-L), 160 kDa (NF-M) and 200 kDa (NF-H). Anti-neurofilament stains a number of neural, neuroendocrine, and endocrine tumors. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas, and neuroblastomas stain positively for anti-neurofilament. Neurofilaments are also present in paragangliomas as well as adrenal and extra-adrenal pheochromocytomas. Carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung also express neurofilament.

Supplier: Biotium
Description: This MAb reacts with a 200 kDa protein, identified as heavy sub-unit of neurofilaments (NF-H). Neurofilaments make up the main structural elements of axons and dendrites and are found in neurons, peripheral nerves, and sympathetic ganglion cells. Neurofilaments consist of three major subunits with molecular weights of 68 kDa (NF-L), 160 kDa (NF-M) and 200 kDa (NF-H). Anti-neurofilament stains a number of neural, neuroendocrine, and endocrine tumors. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas, and neuroblastomas stain positively for anti-neurofilament. Neurofilaments are also present in paragangliomas as well as adrenal and extra-adrenal pheochromocytomas. Carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung also express neurofilament.

Catalog Number: (BOSSBS-7593R-A750)
Supplier: Bioss
Description: Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-4168R-HRP)
Supplier: Bioss
Description: TNIK is a MSN protein kinase that interacts with both TNF receptor-associated factor 2 (TRAF2) and the adapter protein NCK. The protein has been shown to activate the c-Jun N-terminal kinase pathway when over expressed in Phoenix-A cells. TNIK has been shown to phosphorylate gelsolin, the principal intracellular and extracellular actin-severing protein, in vitro. This and evidence from mutational studies suggest that TNIK functions in the regulation of the cytoskeleton. Northern analysis indicates TNIK expression in human heart, skeletal muscle, and brain, with lower levels of expression in kidney, liver, lung, and pancreas. ESTs have been isolated from human tissue libraries, including normal amnion, gallbladder and skin.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-6425R-A488)
Supplier: Bioss
Description: Non-selective calcium permeant cation channel probably involved in osmotic sensitivity and mechanosensitivity. Activation by exposure to hypotonicity within the physiological range exhibits an outward rectification. Also activated by low pH, citrate and phorbol esters. Increase of intracellular Ca(2+) potentiates currents. Channel activity seems to be regulated by a calmodulin-dependent mechanism with a negative feedback mechanism. Promotes cell-cell junction formation in skin keratinocytes and plays an important role in the formation and/or maintenance of functional intercellular barriers. Acts as a regulator of intracellular Ca(2+) in synoviocytes. Plays an obligatory role as a molecular component in the nonselective cation channel activation induced by 4-alpha-phorbol 12,13-didecanoate and hypotonic stimulation in synoviocytes and also regulates production of IL-8.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A555)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-5352R-A680)
Supplier: Bioss
Description: Cytokeratins (CK) are intermediate filaments of epithelial cells, both in keratinising tissue (ie., skin) and non keratinising cells (ie., mesothelial cells). Although not a traditional marker for endothelial cells, cytokeratins have also been found in some microvascular endothelial cells. At least 20 different cytokeratins (CK) in the molecular range of 40 to 70 kDa and isoelectric points of 5 to 8.5 can be identified using two dimensional gel electrophoresis. Biochemically, most members of the CK family fall into one of two classes, type I (acidic polypeptides) and type II (basic polypeptides). At least one member of the acidic family and one member of the basic family is expressed in all epithelial cells. Defects in KRT5 are a cause of epidermolysis bullosa simplex.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-5352R-A750)
Supplier: Bioss
Description: Cytokeratins (CK) are intermediate filaments of epithelial cells, both in keratinising tissue (ie., skin) and non keratinising cells (ie., mesothelial cells). Although not a traditional marker for endothelial cells, cytokeratins have also been found in some microvascular endothelial cells. At least 20 different cytokeratins (CK) in the molecular range of 40 to 70 kDa and isoelectric points of 5 to 8.5 can be identified using two dimensional gel electrophoresis. Biochemically, most members of the CK family fall into one of two classes, type I (acidic polypeptides) and type II (basic polypeptides). At least one member of the acidic family and one member of the basic family is expressed in all epithelial cells. Defects in KRT5 are a cause of epidermolysis bullosa simplex.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-15148R-CY3)
Supplier: Bioss
Description: The second largest human chromosome, 2 consists of 237 million bases encoding over 1,400 genes and making up approximately 8% of the human genome. A number of genetic diseases are linked to genes on chromosome 2. Harlequin icthyosis, a rare and morbid skin deformity, is associated with mutations in the ABCA12 gene. The lipid metabolic disorder sitosterolemia is associated with ABCG5 and ABCG8. An extremely rare recessive genetic disorder, Alstr syndrome is due to mutations in the ALMS1 gene. Interestingly, chromosome 2 contains what appears to be a vestigial second centromere and vestigial telomeres which gives credence to the hypothesis that human chromosome 2 is the result of an ancient fusion of two ancestral chromosomes seen in modern form today in apes. The C2orf27 gene product has been provisionally designated C2orf27 pending further characterization.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-8143R)
Supplier: Bioss
Description: The coiled-coil domain is a structural motif found in proteins that are involved in a diverse array of biological functions such as the regulation of gene expression, cell division, membrane fusion, and drug extrusion and delivery. CCDC93 (coiled-coil domain containing 93) is a 631 amino acid protein that belongs to the CCDC93 family. CCDC93 is encoded by a gene located on human chromosome 2, which makes up approximately 8% of the human genome and contains 237 million bases encoding over 1,400 genes. A number of genetic diseases are linked to genes on chromosome 2. Harlequin icthyosis, a rare skin deformity, is associated with mutations in the ABCA12 gene. The lipid metabolic disorder sitosterolemia is associated with ABCG5 and ABCG8. An extremely rare recessive genetic disorder, Alstré°‰ syndrome, is related to mutations in the ALMS1 gene.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9020R-HRP)
Supplier: Bioss
Description: The second largest human chromosome, 2 consists of 237 million bases encoding over 1,400 genes and making up approximately 8% of the human genome. A number of genetic diseases are linked to genes on chromosome 2. Harlequin icthyosis, a rare and morbid skin deformity, is associated with mutations in the ABCA12 gene. The lipid metabolic disorder sitosterolemia is associated with ABCG5 and ABCG8. An extremely rare recessive genetic disorder, Alstré°‰ syndrome is due to mutations in the ALMS1 gene. Interestingly, chromosome 2 contains what appears to be a vestigial second centromere and vestigial telomeres which gives credence to the hypothesis that human chromosome 2 is the result of an ancient fusion of two ancestral chromosomes seen in modern form today in apes.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-7593R-A555)
Supplier: Bioss
Description: Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
UOM: 1 * 100 µl


Supplier: Biotium
Description: This MAb reacts with a 200 kDa protein, identified as heavy sub-unit of neurofilaments (NF-H). Neurofilaments make up the main structural elements of axons and dendrites and are found in neurons, peripheral nerves, and sympathetic ganglion cells. Neurofilaments consist of three major subunits with molecular weights of 68 kDa (NF-L), 160 kDa (NF-M) and 200 kDa (NF-H). Anti-neurofilament stains a number of neural, neuroendocrine, and endocrine tumors. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas, and neuroblastomas stain positively for anti-neurofilament. Neurofilaments are also present in paragangliomas as well as adrenal and extra-adrenal pheochromocytomas. Carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung also express neurofilament.

Catalog Number: (BOSSBS-11163R-HRP)
Supplier: Bioss
Description: Esophagin, also known as small proline-rich protein 3 (SPR3) or Cornifin ∫, belongs to the cornifin family of cornified-envelope structural proteins. It is expressed in mucosal epithelia such as esophagus and tongue and is strongly induced during epidermal keratinocyte differentiation. Due to its highly inducible nature, Esophagin is considered a marker of squamous differentiation. Esophagin serves as a cross-linking protein within the cornified cell envelope and may play a role in the maintenance of normal esophageal epithelial homeostasis. It shares significant homology with the related proteins, SPRR1 and SPRR2. Esophagin is typically not expressed in healthy human epithelium, but its expression is upregulated in numerous hyperproliferative disorders of the skin. Contrastly, its expression is dramatically downregulated in esophageal squamous cell carcinoma.
UOM: 1 * 100 µl


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