7-Dehydrocholesterol Reductase (DHCR7) (AA 437-463), (C-Term) antibody

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Antigen
Synonyms 7-Dhcr, Xdhcr7, MGC64361, slos, 7-STEROL DELTA7 REDUCTASE, 7RED, DELTA5, DWARF 5, F11F12.21, F11F12_21, LE, LEPIDA, PA, PARVA, ST7R, STEROL DELTA7 REDUCTASE, DKFZp468C1911, AA409147, SLOS, xdhcr7
Epitope
AA 437-463, C-Term
(15), (14), (2), (1)
Reactivity
Mouse (Murine)
(29), (22), (15)
Host
Rabbit
(36)
Clonality (Clone)
Polyclonal ()
Conjugate
Un-conjugated
(3), (3), (3), (2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
(23), (17), (13), (11), (9), (6), (4), (3)
Pubmed 1 reference available
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Quantity 400 μL
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Immunogen This DHCR7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 437-463 AA from the C-terminal region of human DHCR7.
Clone RB17942
Isotype Ig
Specificity This DHCR7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 444-471 amino acids from the C-terminal region of human DHCR7.
Purification This antibody is purified through a protein A column, followed by peptide affinity purification.
Alternative Name DHCR7 (DHCR7 Antibody Abstract)
Background This gene encodes an enzyme that removes the C(7-8) double bond in the B ring of sterols and catalyzes the conversion of 7-dehydrocholesterol to cholesterol. This gene is ubiquitously expressed and its transmembrane protein localizes to the endoplasmic reticulum membrane and nuclear outer membrane. Mutations in this gene cause Smith-Lemli-Opitz syndrome (SLOS), a syndrome that is metabolically characterized by reduced serum cholesterol levels and elevated serum 7-dehydrocholesterol levels and phenotypically characterized by mental retardation, facial dysmorphism, syndactyly of second and third toes, and holoprosencephaly in severe cases to minimal physical abnormalities and near-normal intelligence in mild cases. Alternative splicing results in multiple transcript variants that encode the same protein.
Synonyms: DHCR7,7-dehydrocholesterol reductase,D7SR
Molecular Weight 54489 DA
Gene ID 1717
NCBI Accession NP_001351, NP_001157289
UniProt Q9UBM7
Research Area Cell Structure, Protein Modifications, Metabolism, Phospho-specific antibodies
Application Notes WB = 1:1000, IHC (p) = 1:50-100, IF = 1:10-50
Restrictions For Research Use only
Format Liquid
Concentration 0.31 mg/mL
Buffer PBS with 0.09 % (W/V) sodium azide
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
Expiry Date 6 months
Supplier Images
Immunohistochemistry (IHC) image for anti-7-Dehydrocholesterol Reductase (DHCR7) (AA 437-463), (C-Term) antibody (ABIN655399) DHCR7 Antibody (C-term) (ABIN655399) immunohistochemistry analysis in formalin fixed ...
Background publications Jugessur, Shi, Gjessing et al.: "Maternal genes and facial clefts in offspring: a comprehensive search for genetic associations in two population-based cleft studies from Scandinavia." in: PLoS ONE, Vol. 5, Issue 7, pp. e11493, 2010 (PubMed).

Catalog No. ABIN655399
$324.50
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