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Mouse Anti-FA2H Recombinant Antibody (CBXF-3567) (CBMAB-F2073-CQ)

This product is a mouse antibody that recognizes FA2H. The antibody CBXF-3567 can be used for immunoassay techniques such as: WB.
See all FA2H antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXF-3567
Antibody Isotype
IgG2b
Application
WB

Basic Information

Immunogen
Full length human recombinant protein of human FA2H (NP_077282)
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
Buffer
PBS, pH 7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Fatty Acid 2-Hydroxylase
Introduction
This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia.
Entrez Gene ID
79152
UniProt ID
Q7L5A8
Alternative Names
Fatty Acid 2-Hydroxylase; Spastic Paraplegia 35 (Autosomal Recessive); Fatty Acid Hydroxylase Domain Containing 1; Fatty Acid Alpha-Hydroxylase; FAAH; Fatty Acid Hydroxylase;
Research Area
Catalyzes the hydroxylation of free fatty acids at the C-2 position to produce 2-hydroxy fatty acids, which are building blocks of sphingolipids and glycosphingolipids common in neural tissue and epidermis (PubMed:15337768, PubMed:15863841, PubMed:17355976, PubMed:22517924).

FA2H is stereospecific for the production of (R)-2-hydroxy fatty acids (PubMed:22517924).

Plays an essential role in the synthesis of galactosphingolipids of the myelin sheath (By similarity).

Responsible for the synthesis of sphingolipids and glycosphingolipids involved in the formation of epidermal lamellar bodies critical for skin permeability barrier (PubMed:17355976).

Participates in the synthesis of glycosphingolipids and a fraction of type II wax diesters in sebaceous gland, specifically regulating hair follicle homeostasis (By similarity).

Involved in the synthesis of sphingolipids of plasma membrane rafts, controlling lipid raft mobility and trafficking of raft-associated proteins (By similarity).
Biological Process
Central nervous system myelin maintenance Source: Ensembl
Ceramide biosynthetic process Source: UniProtKB
Establishment of skin barrier Source: UniProtKB
Fatty acid biosynthetic process Source: UniProtKB-KW
Fatty acid metabolic process Source: GO_Central
Galactosylceramide biosynthetic process Source: UniProtKB
Glucosylceramide biosynthetic process Source: UniProtKB
Lipid modification Source: Ensembl
Peripheral nervous system myelin maintenance Source: Ensembl
Plasma membrane raft organization Source: UniProtKB
Regulation of cell population proliferation Source: Ensembl
Regulation of hair cycle Source: Ensembl
Sebaceous gland cell differentiation Source: Ensembl
Sphingolipid biosynthetic process Source: Reactome
Cellular Location
Endoplasmic reticulum membrane; Microsome membrane
Involvement in disease
Spastic paraplegia 35, autosomal recessive (SPG35):
A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG35 is a complicated form characterized by childhood onset of gait difficulties. It has a rapid progression and many patients become wheelchair-bound as young adults. Patients manifest cognitive decline associated with leukodystrophy. Other variable neurologic features, such as dystonia, optic atrophy, and seizures may also occur.

Zhou, X., Huang, F., Ma, G., Wei, W., Wu, N., & Liu, Z. (2022). Dysregulated ceramides metabolism by fatty acid 2-hydroxylase exposes a metabolic vulnerability to target cancer metastasis. Signal Transduction and Targeted Therapy, 7(1), 1-14.

Sakai, G., Hirao-Suzuki, M., Koga, T., Kobayashi, T., Kamishikiryo, J., Tanaka, M., ... & Takeda, S. (2022). Perfluorooctanoic acid (PFOA) as a stimulator of estrogen receptor-negative breast cancer MDA-MB-231 cell aggressiveness: Evidence for involvement of fatty acid 2-hydroxylase (FA2H) in the stimulated cell migration. The Journal of Toxicological Sciences, 47(4), 159-168.

Jordans, S., Hardt, R., Becker, I., Winter, D., Wang-Eckhardt, L., & Eckhardt, M. (2022). Age-Dependent Increase in Schmidt-Lanterman Incisures and a Cadm4-Associated Membrane Skeletal Complex in Fatty Acid 2-hydroxylase Deficient Mice: a Mouse Model of Spastic Paraplegia SPG35. Molecular Neurobiology, 1-11.

Kawaguchi, M., Sassa, T., Kidokoro, H., Nakata, T., Kato, K., Muramatsu, H., ... & Natsume, J. (2020). Novel biallelic FA2H mutations in a Japanese boy with fatty acid hydroxylase-associated neurodegeneration. Brain and Development, 42(2), 217-221.

Qi, T., Wu, D., Duan, Z., Chen, C., Qiu, J., & Kang, J. (2020). Overexpression of Fatty Acid 2-Hydroxylase is Associated with an Increased Sensitivity to Cisplatin by Ovarian Cancer and Better Prognoses. Genetic Testing and Molecular Biomarkers, 24(10), 632-640.

Hirao-Suzuki, M., Koga, T., Sakai, G., Kobayashi, T., Ishii, Y., Miyazawa, H., ... & Takeda, S. (2020). Fatty acid 2-hydroxylase (FA2H) as a stimulatory molecule responsible for breast cancer cell migration. Biochemical and Biophysical Research Communications, 531(2), 215-222.

Hirao-Suzuki, M., Takeda, S., Watanabe, K., Takiguchi, M., & Aramaki, H. (2019). Δ9-Tetrahydrocannabinol upregulates fatty acid 2-hydroxylase (FA2H) via PPARα induction: A possible evidence for the cancellation of PPARβ/δ-mediated inhibition of PPARα in MDA-MB-231 cells. Archives of biochemistry and biophysics, 662, 219-225.

Incecik, F., Besen, S., & Bozdogan, S. T. (2018). Hereditary spastic paraplegia type 35 with a novel mutation in fatty acid 2-hydroxylase gene and literature review of the clinical features. Annals of Indian Academy of Neurology, 21(4), 335.

Hardt, R., Winter, D., Gieselmann, V., & Eckhardt, M. (2018). Identification of progesterone receptor membrane component-1 as an interaction partner and possible regulator of fatty acid 2-hydroxylase. Biochemical Journal, 475(5), 853-871.

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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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