Anti-Human Frataxin Recombinant Antibody (SAA2739) (HS777033)

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100ug
1mg

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Overview

Catalog No.

HS777033

Description

Anti-Human Frataxin Antibody (SAA2739) [SAA2739] (HS777033) is a human monoclonal antibody detecting Frataxin in ELISA. Suitable for Human.

Species reactivity

Human

Applications

ELISA

Host species

Human

Isotype

VHH-Fc

Clone ID

SAA2739

Clonality

Monoclonal

Target

EC:1.16.3.1, Extramitochondrial frataxin, FRDA, FXN, Frataxin intermediate form, Frataxin mature form, Frataxin(56-210), Frataxin(78-210), Frataxin(81-210), Frataxin, mitochondrial, Friedreich ataxia protein, Fxn, X25, d-FXN, i-FXN, m56-FXN, m78-FXN, m81-FXN

Endotoxin level

Please contact with the lab for this information.

Purity

>95% as determined by SDS-PAGE.

Purification

Protein A/G purified from cell culture supernatant.

Accession

Q16595

Form

Liquid

Storage buffer

0.01M PBS pH 7.4

Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA.

Product Usage Information

Application	Dilution
ELISA	1:2000-1:20000

Stability and Storage

Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.

Background

Frataxin, mitochondrial is a ~23 kDa protein. Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly. Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release. Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly. The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner.

1. Yoon, T. et al. (2003) Journal of the American Chemical Society 125, 6078-84. PMID: 12785837

2. Bridwell-Rabb, J. et al. (2014) Biochemistry 53, 4904-13. PMID: 24971490

3. Cai, K. et al. (2018) Journal of inorganic biochemistry 183, 107-116. PMID: 29576242

4. O'Neill, HA. et al. (2005) Biochemistry 44, 537-45. PMID: 15641778

5. Cavadini, P. et al. (2002) Human molecular genetics 11, 217-27. PMID: 11823441

6. Nichol, H. et al. (2003) Biochemistry 42, 5971-6. PMID: 12755598

7. Bulteau, AL. et al. (2004) Science (New York, N.Y.) 305, 242-5. PMID: 15247478

8. Yoon, T. et al. (2004) The Journal of biological chemistry 279, 25943-6. PMID: 15123683

9. Schoenfeld, RA. et al. (2005) Human molecular genetics 14, 3787-99. PMID: 16239244

Note

For research use only

Images

References

Datasheet

Anti-Human Frataxin Recombinant Antibody (SAA2739).pdf

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Formula

Mass (g) = Concentration (mol/L) × Volume (L) × MW (g/mol)

Enter any 2 of Mass, Concentration, Volume + Molecular Weight to solve for the unknown.

Mass

Concentration

Volume

Molecular Weight *

g/mol

Formula

C₁ × V₁ = C₂ × V₂

Enter any 3 of the 4 values to solve for the unknown.

Stock Solution

C₁ (Stock Conc.)

V₁ (Stock Vol.)

Working Solution

C₂ (Working Conc.)

V₂ (Working Vol.)

Recombinant Proteins

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Anti-Human Frataxin Recombinant Antibody (SAA2739) (HS777033)

Overview

Images

References

Datasheet

SDS

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