A1555G-F133

**Figure 1\. Pedigree diagram for family A1555G\-F133\.**

![](/media/202607/A1555G-F133-pedigree_20260701021110913397.png#size=100%&align=left)

# Pedigree Information

| **No.** | **Position** | **Variant** | **Family ID** | **Nationality** | **Diseases** | **Unaffected Maternal Relatives** | **Affected Maternal Relatives** | **Publication Year** | **PMID/Ref** | **Note** |
| --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- |
| 1 | 1555 | m.1555A\>G | A1555G\-F133 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | 0 | 1 | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G144 |

The **m.1555A\>G** variant in MT\-RNR1 was reported in family A1555G\-F133 from Poland with nonsyndromic and aminoglycoside\-induced hearing loss. The pedigree record reported 0 unaffected and 1 affected maternal relatives, and the carrier table includes 2 listed carriers. Homoplasmy was reported in 1/2 listed carriers; 2/2 carriers were affected, and the main clinical manifestation among affected carriers was hearing loss, pta not available, hearing impairment.

# Carrier Information

| **No.** | **Position** | **Variant** | **Family ID** | **Carrier ID** | **Type (Fam/De novo/Uninf)** | **Gender (F/M)** | **Proband (Y/N)** | **Affected (Y/N)** | **Die/Alive(D/A)** | **Age** | **Blood Mutant Level** | **Muscle Mutant level** | **Urine Mutant level** | **Other Mutant level** | **Clinical Features** | **Note** |
| --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- |
| 1 | 1555 | m.1555A\>G | A1555G\-F133 | A1555G\-F133\-I2 | Uninf | F | Y | Y | ND | onset 6 | Homo | / | / | / | Hearing loss; PTA not available | gentamicin exposure; family history yes (1 affected maternal relative) |
| 2 | 1555 | m.1555A\>G | A1555G\-F133 | A1555G\-F133\-II1 | Fam | F | N | Y | ND | ND | / | / | / | / | Hearing impairment | specific phenotype not individualized; by Fig. 1A affected symbol |

For abbreviations and other usage instructions, please see [Mitofam Info](https://mitofam.com/doc/385/).