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The antibody against NSDHL was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human NSDHL (NP_057006.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
The antibody against NSDHL was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human NSDHL (NP_057006.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
| Cat.No | ADA-13367A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | NSDHL |
| Target Synonyms | H105E3; XAP104; SDR31E1; HL | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | 293T | Application | ELISA, WB, IF/ICC, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human NSDHL (NP_057006.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q15738 | Immunogen Sequence |
Uniprot Id
Q15738
Target Species
Human
Target Name
NSDHL
Target Full Name
Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating
Target Function
Catalyzes the NAD(P)(+)-dependent oxidative decarboxylation of the C4 methyl groups of 4-alpha-carboxysterols in post-squalene cholesterol biosynthesis. Plays also a role in the regulation of the endocytic trafficking of EGFR.
Target Involvement
Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD); CK syndrome (CKS)
Target Subcellular Location
Endoplasmic reticulum membrane; Single-pass membrane protein. Lipid droplet.
Target Protein Families
3-beta-HSD family
Target Tissue Specificity
Brain, heart, liver, lung, kidney, skin and placenta.
Target Synonyms
NSDHL; H105E3; Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating; Protein H105e3
Target Background
The protein encoded by this gene is localized in the endoplasmic reticulum and is involved in cholesterol biosynthesis. Mutations in this gene are associated with CHILD syndrome, which is a X-linked dominant disorder of lipid metabolism with disturbed cholesterol biosynthesis, and typically lethal in males. Alternatively spliced transcript variants with differing 5' UTR have been found for this gene.
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