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2-(1-Methylguanidino)ethyl+dihydrogen+phosphate
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2-(1-Methylguanidino)ethyl+dihydrogen+phosphate
Catalog Number:
(10353-048)
Supplier:
Bioss
Description:
Inhibitor of protein-phosphatase 1.
Catalog Number:
(10389-578)
Supplier:
Bioss
Description:
CYP2R1 is a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are mono-oxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This enzyme is a microsomal vitamin D hydroxylase that converts vitamin D into the active ligand for the vitamin D receptor.Defects in CYP2R1 are a cause of 25-hydroxyvitamin D(3) deficiency, also known as pseudovitamin D(3) deficiency rickets due to 25-hydroxylase deficiency. First described in patients who had rickets at a young age despite a history of adequate vitamin D intake. The patients sera had low calcium concentrations, low phosphate concentrations, elevated alkaline phosphatase activity and low levels of 25-hydroxyvitamin D.
Catalog Number:
(10750-190)
Supplier:
Prosci
Description:
LIMP2 Antibody: The lysosomal integral membrane protein 2 (LIMP2) is a heavily glycosylated type III transmembrane protein, the majority of which exists in the lumen of the lysosome and a cytoplasmic domain of approximately 20 amino acids. A deficiency of LIMP2 in mice causes uretic pelvic junction obstruction, deafness, and peripheral neuropathy associated with impaired vesicular trafficking and distribution of apically expressed proteins. More recently, LIMP2 was shown to act as a receptor to bind beta-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted beta-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor.
Catalog Number:
(10665-932)
Supplier:
Bioss
Description:
Pyridoxal kinase belongs to the pyridoxine kinase family and phosphorylates vitamin B6, a step necessary for the conversion of vitamin B6 to pyridoxal 5’ phosphate (PLP), the active form of vitamin B6. PLP acts as a coenzyme and functions to maintain homeostasis. Pyridoxal kinase is a 312-amino acid cytoplasmic protein that may act as a homodimer and is expressed ubiquitously. There are three known isoforms of pyridoxal kinase, and isoform 3 expression is observed in adult testis and spermatozoa. The optimum pH for pyridoxal kinase is between 5.5 and 6.0. PDXK, the gene that encodes the pyridoxal kinase protein, maps to chromosome 21q22.3 and may be a candidate gene for autoimmune polyglandular disease type 1, a genetic disorder that has been mapped to the same region on chromosome 21.
Catalog Number:
(10099-970)
Supplier:
Prosci
Description:
POLK belongs to the DNA polymerase type-Y family. It contains 2 Rad18-type zinc fingers and 1 umuC domain. POLK is a DNA polymerase specifically involved in DNA repair. It plays an important role in translesion synthesis, where the normal high-fidelity DNA polymerases cannot proceed and DNA synthesis stalls. Depending on the context, it inserts the correct base, but causes frequent base transitions, transversions and frameshifts. It lacks 3'-5' proofreading exonuclease activity. POLK forms a Schiff base with 5'-deoxyribose phosphate at abasic sites, but does not have lyase activity.
Catalog Number:
(10164-982)
Supplier:
Genetex
Description:
Rabbit Polyclonal antibody to SPP24
Supplier:
TCI America
Description:
CAS Number: 6757-06-8
MDL Number: MFCD00064355 Molecular Formula: C9H14N3O8P Molecular Weight: 367.16 Purity/Analysis Method: >99.0% (HPLC) Form: Crystal Specific rotation [a]20/D: 8 deg (C=2, H2O) Storage Temperature: 0-10°C
Catalog Number:
(10748-722)
Supplier:
Prosci
Description:
LIMP2 Antibody: The lysosomal integral membrane protein 2 (LIMP2) is a heavily glycosylated type III transmembrane protein, the majority of which exists in the lumen of the lysosome and a cytoplasmic domain of approximately 20 amino acids. A deficiency of LIMP2 in mice causes uretic pelvic junction obstruction, deafness, and peripheral neuropathy associated with impaired vesicular trafficking and distribution of apically expressed proteins. More recently, LIMP2 was shown to act as a receptor to bind beta-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted beta-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor.
Catalog Number:
(10264-016)
Supplier:
Bioss
Description:
The Huntingtin protein contains a polyglutamine region, which leads to Huntingtin’s disease (HD) when the number of glutamine repeats exceeds thirty-five. The mutated Huntingtin protein acts within the nucleus to induce neurodegeneration by a cell-specific apoptotic mechanism. The loss of activity of the Huntingtin protein may be contributed to abnormal interactions between the mutant protein and other associated cellular proteins. Huntingtin interacts with a variety of proteins including HAP1, glyceraldehyde phosphate dehydrogenase (GAPDH), HAP40, Rab5 and HIP1. HAP40 mediates the recruitment of Huntingtin by Rab5 onto early endosomes. Specifically, this complex regulates endosome motility, which may be a key event of the pathogenetic process leading to neurodegeneration in HD.
Catalog Number:
(10482-262)
Supplier:
Bioss
Description:
The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
Catalog Number:
(RL610-603-002)
Supplier:
Rockland Immunochemical
Description:
Suitable for immunoblotting (western or dot blot), ELISA, immunoperoxidase electron microscopy and immunochemistry as well as other peroxidase-antibody based enzymatic assays requiring lot-to-lot consistency.
Catalog Number:
(89270-130)
Supplier:
Genetex
Description:
Rabbit polyclonal antibody to GFPT2 (Middle)
Catalog Number:
(103272-424)
Supplier:
Novus Biologicals
Description:
The CPS1 Antibody from Novus Biologicals is a rabbit polyclonal antibody to CPS1. This antibody reacts with human, canine. The CPS1 Antibody has been validated for the following applications: Western Blot, Immunohistochemistry, Immunocytochemistry / Immunofluorescence, Immunohistochemistry-Paraffin.
Catalog Number:
(103332-716)
Supplier:
Novus Biologicals
Description:
The SLC25A3 Antibody from Novus Biologicals is a mouse polyclonal antibody to SLC25A3. This antibody reacts with human. The SLC25A3 Antibody has been validated for the following applications: Western Blot.
Catalog Number:
(89292-330)
Supplier:
Genetex
Description:
Rabbit Polyclonal antibody to CI-MPR
Catalog Number:
(RL705-4313)
Supplier:
Rockland Immunochemical
Description:
This product has been assayed against 1.0 µg of Goat IgG in a standard capture ELISA using ABTS (2,2’-azino-bis-[3-ethylbenthiazoline-6-sulfonic acid])
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 is still displayed and you need assistance, please call us at 1-800-932-5000.
Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the
is still displayed and you need assistance, please call us at 1-800-932-5000.
This product is marked as restricted and can only be purchased by approved Shipping Accounts. If you need further assistance, email VWR Regulatory Department at Regulatory_Affairs@vwr.com
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