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Rabbit Anti-DEGS1 Recombinant Antibody (D0695) (CBMAB-D0695-YC)

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Summary

Host Animal
Rabbit
Specificity
Human
Clone
D0695
Antibody Isotype
IgG
Application
ELISA, WB

Basic Information

Immunogen
Recombinant protein amino acids 1-100 of human MLD.
Host Species
Rabbit
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
ELISA1 µg/ml
WB1:500-1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
BSA & Glycerol & PBS
Preservative
Sodium Azide
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 226-323

Target

Full Name
degenerative spermatocyte homolog 1, lipid desaturase (Drosophila)
Introduction
DEGS1 belongs to the membrane fatty acid desaturase family which is responsible for inserting double bonds into specific positions in fatty acids. This protein contains three His-containing consensus motifs that are characteristic of a group of membrane fatty acid desaturases. It is predicted to be a multiple membrane-spanning protein localized to the endoplasmic reticulum. Overexpression of this gene inhibited biosynthesis of the EGF receptor, suggesting a possible role of a fatty acid desaturase in regulating biosynthetic processing of the EGF receptor.
Entrez Gene ID
UniProt ID
Alternative Names
Delta 4-Desaturase, Sphingolipid 1; Cell Migration-Inducing Gene 15 Protein; Sphingolipid Delta(4)-Desaturase 1; Dihydroceramide Desaturase 1; Membrane Lipid Desaturase; DES1; MLD; Degenerative Spermatocyte Homolog 1, Lipid Desaturase (Drosophila); Degenerative Spermatocyte Homolog 1, Lipid Desaturase; Degenerative Spermatocyte Homolog, Lipid Desaturase; Membrane Fatty Acid (Lipid) Desaturase;
Function
Has sphingolipid-delta-4-desaturase activity. Converts D-erythro-sphinganine to D-erythro-sphingosine (E-sphing-4-enine) (PubMed:11937514, PubMed:30620337, PubMed:30620338).

Catalyzes the equilibrium isomerization of retinols (By similarity).
Biological Process
Ceramide biosynthetic process Source: GO_Central
Myelin maintenance Source: UniProtKB
Neutrophil degranulation Source: Reactome
Dphingolipid biosynthetic process Source: Reactome
Unsaturated fatty acid biosynthetic process Source: ProtInc
Cellular Location
Endoplasmic reticulum membrane; Mitochondrion membrane
Involvement in disease
Leukodystrophy, hypomyelinating, 18 (HLD18):
An autosomal recessive disorder characterized by hypomyelinating leukodystrophy with progressive atrophy of the corpus callosum, thalami and cerebellum, and peripheral neuropathy. Clinical features include very poor psychomotor development, dystonia, severe spasticity, seizures, and failure to thrive.
PTM
Myristoylation can target the enzyme to the mitochondria leading to an increase in ceramide levels.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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