MAB2164
Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
ascites fluid, clone 2AL-1D6, Chemicon®
Synonym(e):
ALDP
Anmeldenzur Ansicht organisationsspezifischer und vertraglich vereinbarter Preise
Alle Fotos(1)
About This Item
UNSPSC-Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Empfohlene Produkte
Biologische Quelle
mouse
Qualitätsniveau
Antikörperform
ascites fluid
Antikörper-Produkttyp
primary antibodies
Klon
2AL-1D6, monoclonal
Speziesreaktivität
human
Hersteller/Markenname
Chemicon®
Methode(n)
ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable
Isotyp
IgG1
NCBI-Hinterlegungsnummer
UniProt-Hinterlegungsnummer
Versandbedingung
dry ice
Posttranslationale Modifikation Target
unmodified
Angaben zum Gen
human ... ABCD1(215)
Spezifität
Human ALDP. No cross reactivity with mouse ALDRP, PMP70. Cross reactivity to an unidentified 80 kD MW protein is present in lymphoblastoid cells, but not in several other cells or tissues tested. MAB2164 can be used to analyze ALDP protein in cells and tissues from normal individuals, or from patients with adrenoleukodystrophy (ALD) or Zellweger syndrome and related peroxisomal diseases, and provides an excellent marker for peroxisomes (Mosser et al. 1994). About 70% of ALD male patients have no detectable ALDP in fibroblasts or leukocytes. A similar proportion of carrier females may show mosaïcism for the presence or absence of ALDP.
Immunogen
ALDP1 fragment from aa 279 to 482 as a fusion protein
Epitope: a.a. 279-482
Anwendung
Research Category
Neurowissenschaft
Neurowissenschaft
Research Sub Category
Neurodegenerative Krankheiten
Neurodegenerative Krankheiten
Detect Adrenoleukodystrophy Protein using this Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 validated for use in ELISA, WB, IC, IH.
ELISA: 1:500-1:5,000
Immunoblotting: 1:500-1:5,000
Immunohistochemistry: 1:500-1:5,000
Immunocytochemistry: 1:500-1:5,000
Optimal working dilutions must be determined by the end user.
Immunoblotting: 1:500-1:5,000
Immunohistochemistry: 1:500-1:5,000
Immunocytochemistry: 1:500-1:5,000
Optimal working dilutions must be determined by the end user.
Physikalische Form
Ascites. Liquid, does not contain any preservative.
Lagerung und Haltbarkeit
Maintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles.
Rechtliche Hinweise
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
Haftungsausschluss
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Lagerklassenschlüssel
10 - Combustible liquids
WGK
nwg
Flammpunkt (°F)
Not applicable
Flammpunkt (°C)
Not applicable
Analysenzertifikate (COA)
Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.
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M Contreras et al.
Archives of biochemistry and biophysics, 334(2), 369-379 (1996-10-15)
Adrenoleukodystrophy (X-ALD) is a demyelinating disorder characterized by the accumulation of saturated very-long-chain fatty acids (> C22:0) due to the impaired activity of lignoceroyl-CoA ligase. The gene responsible for the disease was found to code for a 84-kDa peroxisomal integral
Miguel Agustin Contreras et al.
Archives of biochemistry and biophysics, 477(2), 211-218 (2008-07-08)
Krabbe disease is a neuroinflammatory disorder in which galactosylsphingosine (psychosine) accumulates in nervous tissue. To gain insight into whether the psychosine-induced effects in nervous tissue extend to peripheral organs, we investigated the expression of cytokines and their effects on peroxisomal
Stefan Schönberger et al.
Archives of neurology, 64(5), 651-657 (2007-03-14)
X-linked adrenoleukodystrophy (X-ALD) is the most common inherited peroxisomal disorder. It is caused by impaired function of ALD protein that results in accumulation of very long-chain fatty acids in tissues and body fluids. So far, hematopoietic stem cell transplantation (HSCT)
J Mosser et al.
Human molecular genetics, 3(2), 265-271 (1994-02-01)
Adrenoleukodystrophy is a severe genetic demyelinating disease associated with an impairment of beta-oxidation of very long chain fatty acids (VLCFA) in peroxisomes. Earlier studies had suggested that a deficiency in VLCFA CoA synthetase was the primary defect. A candidate adrenoleukodystrophy
X-linked adrenoleukodystrophy.
Aubourg, P and Mandel, J L
Annals of the New York Academy of Sciences, 804, 461-476 (1996)
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